The Cardiovascular Preparticipation Evaluation (PPE)

SPECIAL COMMUNICATIONS The Cardiovascular Preparticipation Evaluation (PPE) for the Primary Care and Sports Medicine Physician, Part I Editors: Irfan M. Asif, MD; William O. Roberts, MD, MS, FACSM; Michael Fredericson, MD, FACSM; and Vic Froelicher, MD Purpose: To provide a rational approach to positive responses to the American Heart Association (AHA) 12-Step Questionnaire and fourth-edition ‘‘Preparticipation Physical Evaluation’’ (PPE) monograph for assessing cardiovascular (CV) risk in athletes. This will assist primary care and sports medicine physicians in determining the need for the following:

1. Follow-up questions to a positive response that will enhance the history and help determine whether a condition that puts an athlete at increased CV risk exists 2. Any basic diagnostic tests to further assess the athlete and that will assist with making an informed decision 3. The need for a consultation or referral to an appropriate specialist Our goal is to help the primary care and sports medicine physician with the critical decision making regarding positive responses to the AHA 12-Step Questionnaire and criteria for athlete clearance, as follows:

1. Could this be a potentially lethal problem? 2. Does this need additional workup or just an electrocardiogram? 3. Does this require consultation with a specialist (and which specialty)? For example, to address a positive response to the question regarding ‘‘excessive shortness of breath or fatigue with exercise beyond what is expected for your level of fitness,’’ it would be useful for physicians to know which elements in the history, physical, or diagnostic tests point to a potentially lethal CV diagnosis versus an easily treated pulmonary issue like exercise-induced asthma. If a lethal diagnosis can be excluded, the responsible physician may be able to determine that no restriction is warranted and clear the athlete for appropriate activity without a referral to a cardiologist or another specialist. While there are some differences in the questions from the AHA 12 points and the CV questions in the PPE fourthedition monograph, the underlying intent is the same and the information provided is easily utilized for both question sets.

History and Application of the AHA 12 Points for Assessing Cardiovascular Risk in Athletes Abhimanyu (Manu) Uberoi, MD, MS and William O. Roberts, MD, MS The cardiovascular (CV) evaluation, one important part of the preparticipation physical examination (PPE), is the 246

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focus of this special communication. Cardiac events during sporting events, albeit rare, can be fatal, and these events are often very public (5,7,10). In the United States, most athlete PPE for ages 6 to 24 years are performed by family physicians and pediatricians (8), some with subspecialty training in sports medicine. Often, the PPE is the first encounter with the health care system for adolescents and serves as the sole opportunity for general screening, risk factor evaluation, and health education. This may be especially true for adolescents in lower income strata. The PPE is intended to reduce the risk of adverse outcomes without unduly restricting athlete participation. A thorough history examination can uncover a large portion of the athlete’s risk for injury or illness, and the physical examination unveils other abnormalities. There are very few proven screening methods that assure an athlete’s health, but the PPE provides a framework to assess and stratify sport participation risk. The intent of these evaluations is to deliver to health care providers pertinent information to educate athletes and parents and enable them to make an informed participation decision. The first PPE monograph was published in 1992 by five organizations (American Academy of Family Physicians, American Academy of Pediatrics, American Medical Society for Sports Medicine, American Orthopedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine). The American College of Sports Medicine joined for the third edition in 2005, and the fourth edition was published in 2010 (1). The American Heart Association (AHA) developed CV preparticipation screening recommendations for young athletes in 1996 and updated the statement in 2007 (8). The AHA and the American College of Cardiology have reaffirmed their position regarding the CV PPE and electrocardiography (ECG) screening in healthy 12- to 25-year-old young people with a comprehensive review that endorses the 12-element history and physical examination in the 2014 Scientific Statement (9). This recent document added two elements regarding palpitations and previous evaluations similar to those in the fourth PPE. The question sets from the two examination recommendations are similar, and the fourth PPE monograph uses the same general questions, with some differences in syntax and depth of question content. The question wording of the third PPE monograph was based on input from parent and high school athlete focus group sessions to enhance the ‘‘understandability’’ of the questions for the end users. Of note, the question sets are based on expert opinion and have not been subjected to scientific study. In the late 1990s, after surveys showed poor compliance with both the use of consensus-based forms and the AHA question set, some high schools and colleges across the country incorporated the elements of the PPE and the AHA Cardiovascular Preparticipation Evaluation

Copyright © 2015 by the American College of Sports Medicine. Unauthorized reproduction of this article is prohibited.

The 12-Step Questionnaire Recommendations for Health Care Providers for Preparticipation Cardiovascular Screening in Athletes (8)

Medical history/personal history 1. Have you ever experienced chest pain or discomfort with exercise? 2. Have you ever nearly lost or actually lost consciousness? 3. Have you ever had excessive shortness of breath or fatigue with exercise beyond what is expected for your level of fitness? 4. Have you ever been told you had a heart murmur? 5. Have you had high blood pressure? Family history 6. Has anyone in your family younger than 50 years of age died suddenly or unexpectedly from heart disease? 7. Has anyone in your family younger than 50 years of age been disabled from heart disease or had cardiac treatments including surgery? 8. Does anyone in your family have hypertrophic or dilated cardiomyopathy, long QT syndrome or other channelopathies, Marfan syndrome, or arrhythmia problem(s)? Physical examination 9. Is a heart murmur present? 10. Are the femoral pulses weak? 11. Does the athlete exhibit the physical findings of Marfan syndrome? 12. Is the cuff blood pressure elevated or uneven between arms? The Preparticipation Physical Evaluation (edition 4) Questions (10) 1. Has a doctor ever denied or restricted your participation in sports for any reason? Heart health questions about you 2. Have you ever passed out or nearly passed out DURING or AFTER exercise? 3. Have you ever had discomfort, pain, tightness, or pressure in your chest during exercise? 4. Does your heart ever race or skip beats (irregular beats) during exercise? 5. Has a doctor ever told you that you have any heart problems? If so, check all that apply: ) High blood pressure ) A heart murmur ) High cholesterol ) A heart infection ) Kawasaki disease Other: ______________________ 6. Has a doctor ever ordered a test for your heart? (For example, ECG/EKG, echocardiogram.) 7. Do you get lightheaded or feel more short of breath than expected during exercise? 8. Have you ever had an unexplained seizure? 9. Do you get more tired or short of breath more quickly than your friends during exercise? Heart health questions about your family 10. Has any family member or relative died of heart problems or had an unexpected or unexplained sudden death before age 50 (including drowning, unexplained car accident, or sudden infant death syndrome)? 11. Does anyone in your family have hypertrophic cardiomyopathy, Marfan syndrome, arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, short QT syndrome, Brugada syndrome, or catecholaminergic polymorphic ventricular tachycardia? 12. Does anyone in your family have a heart problem, pacemaker, or implanted defibrillator? 13. Has anyone in your family had unexplained fainting, unexplained seizures, or near drowning? Physical examination BP / ( / ) pulse Appearance

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&Marfan stigmata (kyphoscoliosis, high-arched palate, pectus excavatum, arachnodactyly, arm span > height, hyperlaxity, myopia, mitral valve prolapse, aortic insufficiency) Heart &Murmurs (auscultation standing, supine, +/j Valsalva) &Location of point of maximal impulse (PMI) Pulses &Simultaneous femoral and radial pulses

questions (3,9) in their athlete evaluations. Despite progress, however, a 2013 Washington state survey showed that only 47% of physicians understood the AHA guidelines (4). This is unfortunate, given the evidence that supports the efficacy of adopting a standardized PPE form (10). Understanding and utilizing the AHA 12-point CV examination and its constellation of responses is essential to make logical, cost-effective decisions regarding additional evaluations, consultations, and sport participation recommendations (1,9). This document is intended to direct the use of diagnostic testing, including ECG, and reduce reflex use of consults and diagnostics along the lines of the American Medical Association ‘‘Choosing Wisely’’ campaign (6) and encourage the implementation of either the AHA 12-point or the PPE-4 CV screening tools as the minimal standard for athlete screening, not to endorse one questionnaire over the other. The AHA 12-point question/examination set and the fourthedition PPE monograph 13 history questions and five physical examination points are outlined as follows. Both emphasize the importance of personal and family history. This Special Communication will provide the reader, and particularly primary care providers conducting the PPE, a framework to address positive responses to questions and findings reveled during the examination. This will help reduce the risk of adverse outcomes and allow athletes to participate in activities that best fit their risk profile. Positive responses to the CV questions require additional history and sometimes diagnostic investigations to rule out CV problems that put athletes at undo risk. Individual questions will be highlighted in a case presentation to illustrate the significance of each question. Informed analysis of the questions and judicious use of diagnostics will be the key to risk reduction, cost control, and unnecessary athlete restriction. The burden of these decisions rests on the shoulders of the physicians performing the PPE. References 1. Bernhardt D, Roberts W. PPE Preparticipation Physical Evaluation. 4th ed. Elk Grove Village (IL): American Academy of Pediatrics; 2012. 2. Cassel CK, Guest JA. Choosing wisely: helping physicians and patients make smart decisions about their care. JAMA. 2012; 307:1801Y2. 3. Glover DW, Maron BJ. Profile of preparticipation cardiovascular screening for high school athletes. JAMA. 1998; 279:1817Y9. 4. Gomez JE, Lantry BR, Saathoff KN. Current use of adequate preparticipation history forms for heart disease screening of high school athletes. Arch. Pediatr. Adolesc. Med. 1999; 153:723Y6. 5. Harmon KG, Asif IM, Klossner D, Drezner JA. Incidence of sudden cardiac death in National Collegiate Athletic Association athletes. Circulation. 2011; 123:1594Y600. 6. Madsen NL, Drezner JA, Salerno JC. Sudden cardiac death screening in adolescent athletes: an evaluation of compliance with national guidelines. Br. J. Sports Med. 2013; 47:172Y7.

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7. Maron BJ, Haas TS, Ahluwalia A, Rutten-Ramos SC. Incidence of cardiovascular sudden deaths in Minnesota high school athletes. Heart Rhythm. 2013; 10:374Y7. 8. Maron BJ, Thompson PD, Ackerman MJ, et al. Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardiology Foundation. Circulation. 2007; 115:1643Y455. 9. Maron BJ, Friedman RA, Kligfield P, et al. Assessment of the 12-lead electrocardiogram as a screening test for detection of cardiovascular disease in healthy general populations of young people (12Y25 years of age): a scientific statement from the American Heart Association and the American College of Cardiology. Circulation. 2014; 130:1303Y34. 10. Roberts WO, Stovitz SD. Incidence of sudden cardiac death in Minnesota high school athletes 1993Y2012 screened with a standardized preparticipation evaluation. J. Am. Coll. Cardiol. 2013; 62:1298Y301.

Chest Pain in Athletes from Personal History Section (Medical Causes) Meagan M. Wasfy, MD and Aaron Baggish, MD 1B). Personal History: Have you ever experienced chest pain or discomfort with exercise? Although cardiac causes of chest pain (Table 1) are relatively uncommon (G6%) in athletes G35 years old, underlying causal conditions include potentially life-threatening diseases such as coronary artery anomalies and hypertrophic cardiomyopathy (HCM) (5). Older athletes with exertional chest discomfort must be assumed to have atherosclerotic coronary disease until proven otherwise. Noncardiac medical and musculoskeletal causes of exertional chest pain are relevant across the age spectrum. Noncardiac medical causes are outlined in Table 2, and musculoskeletal causes are discussed in the following section (part 3). The key components of a comprehensive medical chest pain history are discussed as follows. What Is the Quality and Location of the Chest Discomfort? Exertional chest discomfort is the most common manifestation of myocardial ischemia, an imbalance of myocardial oxygen demand and supply. Ischemic chest pain can result from a number of cardiac conditions including coronary pathologies (i.e., coronary anomalies, atherosclerotic coronary disease) that limit myocardial blood flow and noncoronary conditions including cardiomyopathy and valvular heart disease that increase myocardial demand. Regardless of the etiology, ischemic chest discomfort is typically 1) reproducibly triggered by effort, 2) relieved by rest, 3) and typically located in the substernal and/or the left chest area. It often Cardiovascular Preparticipation Evaluation


&Marfan stigmata (kyphoscoliosis, high-arched palate, pectus excavatum, arachnodactyly, arm span > height, hyperlaxity, myopia, mitral valve prolapse, aortic insufficiency) Heart &Murmurs (auscultation standing, supine, +/j Valsalva) &Location of point of maximal impulse (PMI) Pulses &Simultaneous femoral and radial pulses

questions (3,9) in their athlete evaluations. Despite progress, however, a 2013 Washington state survey showed that only 47% of physicians understood the AHA guidelines (4). This is unfortunate, given the evidence that supports the efficacy of adopting a standardized PPE form (10). Understanding and utilizing the AHA 12-point CV examination and its constellation of responses is essential to make logical, cost-effective decisions regarding additional evaluations, consultations, and sport participation recommendations (1,9). This document is intended to direct the use of diagnostic testing, including ECG, and reduce reflex use of consults and diagnostics along the lines of the American Medical Association ‘‘Choosing Wisely’’ campaign (6) and encourage the implementation of either the AHA 12-point or the PPE-4 CV screening tools as the minimal standard for athlete screening, not to endorse one questionnaire over the other. The AHA 12-point question/examination set and the fourthedition PPE monograph 13 history questions and five physical examination points are outlined as follows. Both emphasize the importance of personal and family history. This Special Communication will provide the reader, and particularly primary care providers conducting the PPE, a framework to address positive responses to questions and findings reveled during the examination. This will help reduce the risk of adverse outcomes and allow athletes to participate in activities that best fit their risk profile. Positive responses to the CV questions require additional history and sometimes diagnostic investigations to rule out CV problems that put athletes at undo risk. Individual questions will be highlighted in a case presentation to illustrate the significance of each question. Informed analysis of the questions and judicious use of diagnostics will be the key to risk reduction, cost control, and unnecessary athlete restriction. The burden of these decisions rests on the shoulders of the physicians performing the PPE. References 1. Bernhardt D, Roberts W. PPE Preparticipation Physical Evaluation. 4th ed. Elk Grove Village (IL): American Academy of Pediatrics; 2012. 2. Cassel CK, Guest JA. Choosing wisely: helping physicians and patients make smart decisions about their care. JAMA. 2012; 307:1801Y2. 3. Glover DW, Maron BJ. Profile of preparticipation cardiovascular screening for high school athletes. JAMA. 1998; 279:1817Y9. 4. Gomez JE, Lantry BR, Saathoff KN. Current use of adequate preparticipation history forms for heart disease screening of high school athletes. Arch. Pediatr. Adolesc. Med. 1999; 153:723Y6. 5. Harmon KG, Asif IM, Klossner D, Drezner JA. Incidence of sudden cardiac death in National Collegiate Athletic Association athletes. Circulation. 2011; 123:1594Y600. 6. Madsen NL, Drezner JA, Salerno JC. Sudden cardiac death screening in adolescent athletes: an evaluation of compliance with national guidelines. Br. J. Sports Med. 2013; 47:172Y7.

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7. Maron BJ, Haas TS, Ahluwalia A, Rutten-Ramos SC. Incidence of cardiovascular sudden deaths in Minnesota high school athletes. Heart Rhythm. 2013; 10:374Y7. 8. Maron BJ, Thompson PD, Ackerman MJ, et al. Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardiology Foundation. Circulation. 2007; 115:1643Y455. 9. Maron BJ, Friedman RA, Kligfield P, et al. Assessment of the 12-lead electrocardiogram as a screening test for detection of cardiovascular disease in healthy general populations of young people (12Y25 years of age): a scientific statement from the American Heart Association and the American College of Cardiology. Circulation. 2014; 130:1303Y34. 10. Roberts WO, Stovitz SD. Incidence of sudden cardiac death in Minnesota high school athletes 1993Y2012 screened with a standardized preparticipation evaluation. J. Am. Coll. Cardiol. 2013; 62:1298Y301.

Chest Pain in Athletes from Personal History Section (Medical Causes) Meagan M. Wasfy, MD and Aaron Baggish, MD 1B). Personal History: Have you ever experienced chest pain or discomfort with exercise? Although cardiac causes of chest pain (Table 1) are relatively uncommon (G6%) in athletes G35 years old, underlying causal conditions include potentially life-threatening diseases such as coronary artery anomalies and hypertrophic cardiomyopathy (HCM) (5). Older athletes with exertional chest discomfort must be assumed to have atherosclerotic coronary disease until proven otherwise. Noncardiac medical and musculoskeletal causes of exertional chest pain are relevant across the age spectrum. Noncardiac medical causes are outlined in Table 2, and musculoskeletal causes are discussed in the following section (part 3). The key components of a comprehensive medical chest pain history are discussed as follows. What Is the Quality and Location of the Chest Discomfort? Exertional chest discomfort is the most common manifestation of myocardial ischemia, an imbalance of myocardial oxygen demand and supply. Ischemic chest pain can result from a number of cardiac conditions including coronary pathologies (i.e., coronary anomalies, atherosclerotic coronary disease) that limit myocardial blood flow and noncoronary conditions including cardiomyopathy and valvular heart disease that increase myocardial demand. Regardless of the etiology, ischemic chest discomfort is typically 1) reproducibly triggered by effort, 2) relieved by rest, 3) and typically located in the substernal and/or the left chest area. It often Cardiovascular Preparticipation Evaluation


has an aching, pressure, or tightness quality and may radiate to the jaw, neck, and/or arm. Nonischemic causes of chest discomfort often present with disease-specific patterns. Aortic dissection is typically described as a tearing pain that starts suddenly, often during intense isometric activity or body collision and radiates to the back. Pain caused by pericarditis is commonly noticed both at resting and during exertion and is typically worsened by lying supine and relieved by standing or sitting forward. Pleural causes of chest pain including pleurisy and pneumothorax are often sharp in nature and exacerbated by deep inspiration. Are There Specific Provoking Factors aside from Exercise Intensity That Are Related to Your Chest Discomfort? Exertional chest discomfort provoked by climate extremes or seasonal allergens is more likely a function of reactive airway disease and not myocardial ischemia. Exertional chest discomfort that occurs immediately after eating or dietary indiscretions may point to a gastrointestinal (GI) cause but can be ischemic. Paroxysmal symptoms of chest tightness that start and stop suddenly and are accompanied by palpitations may be due to exercise-induced arrhythmia. When during the Course of Exercise Does Your Chest Discomfort Occur? Chest pain caused by myocardial ischemia is typically effort dependent and occurs reproducibly at a given workload. Athletes with ischemic forms of cardiovascular disease may be able to exercise for long periods of time at low-tomoderate workloads without symptoms. Athletes with atherosclerotic coronary disease, a disease process that is increasingly relevant to youthful populations, may experience ‘‘warm-up angina,’’ a term used to describe symptoms that develop during the first few minutes of exercise and then abate despite continued or increased workload. Athletes with an anomalous coronary artery may experience unpredictable and irregular episodes of ischemic exertional chest discomfort. An athlete presenting with sporadic and infrequent exertional chest discomfort occurring only at near-maximal exercise intensity should be studied to rule out this diagnosis. How Long Does It Take for Your Chest Discomfort To Resolve after Stopping Exercise? Chest discomfort due to myocardial ischemia will typically abate within several minutes of either rest or reduced exercise intensity. In contrast, symptoms caused by reactive airway disease or gastroesophageal reflux disease (GERD) more commonly persist for many minutes to hours. Chest symptoms caused by an underlying cardiac arrhythmia typically track with the onset and termination of the arrhythmia and may suddenly resolve without changes in exercise intensity. Have You Ever Had Syncope or Near-Syncope with Your Chest Discomfort? Syncope or lightheadedness that occurs with exertional chest discomfort is a clinical red flag for a potentially lifethreatening condition. The combination of chest discomfort and near-syncope suggests that there is transient impairment www.acsm-csmr.org

of cardiac function that is sufficient to reduce delivery of blood to the brain. Athletes with this symptom constellation should refrain from exercise until comprehensive clinical investigation is completed. Are There Any Other Symptoms That Accompany Your Exertional Chest Discomfort? Exertional chest discomfort caused by a paroxysmal tachyarrhythmia is often preceded and/or accompanied by the sensation of palpitations or a ‘‘racing heart.’’ Pulmonary causes of chest discomfort such as exercise-induced asthma or vocal cord dysfunction are commonly associated with cough, dyspnea, wheeze, or stridor that may be audible to the athlete or others. GI etiologies for chest pain such as GERD characteristically cause epigastric discomfort than may be related to the timing of food intake or dietary indiscretions. GERD may be associated with heartburn, a sour taste in the mouth, dysphagia, dry cough, hoarseness, sore throat, regurgitation of stomach contents, hoarseness, and/or nausea. Chest pain that occurs both at rest and during exertion coupled with systemic symptoms such as malaise, fever, or arthralgia should raise concern for inflammatory processes affecting the pericardium (pericarditis), the myocardium (myocarditis), or the lungs (pneumonia and pleurisy). Has Discomfort in Your Chest Ever Caused You To Slow Down and/or Reduce Your Training Regimen? Athletes with underlying cardiovascular diseases that cause exertional chest discomfort often learn to ‘‘avoid the symptoms.’’ Through trial and error, often subconsciously, they learn to complete personally meaningful levels of daily exercise but remain below their ischemic threshold. Careful review of an athlete’s training regimen and competition history can uncover this adaptation. Has Anyone in Your Immediate or Extended Family Experienced Sudden Cardiac Death, Unexplained Death or Collapse, Been Diagnosed with an Inherited or Congenital Heart Condition, Been Restricted from Competitive Athletics, or Received an Implantable Cardiac Defibrillator? Many important underlying causes of exertional heart disease are genetic and thus inherited. We therefore advocate careful interrogation of family history in all athletic patients with exertional chest discomfort. It is valuable to go beyond simply asking the athlete if there is any family history of heart conditions by inquiring about the specific issues. If any uncertainty is encountered in the response to these questions, further acquisition of medical records and direct communication with the family are warranted. What Medications, Supplements, Illicit Drugs, or Performance-Enhancing Agents Do You Use? Use of performance-enhancing agents (PEA) is increasingly common among athletes and should be queried in all athletic patients presenting for medical care. Anabolic-androgenic steroids are the most widely used PEA and have been previously shown to have deleterious cardiovascular effects causing dyslipidemia, exaggerated blood pressure response to exercise, and myocardial dysfunction (2Y4). The use of erythropoietin analogs, while most commonly associated with central Current Sports Medicine Reports


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nervous system vascular dysfunction, can precipitate microvascular coronary artery infarction from excessive red cell mass. The use of prescription, over-the-counter, or herbal stimulants is increasingly common among competitive athletes. While their impact on cardiac health remains largely unknown, anecdotal experience suggests that their use may precipitate subjective cardiac symptoms and/or unmask previously occult cardiac disease. It is recommended that a comprehensive exertional chest pain history include direct inquiry about exposure to all of the mentioned agents. CASE REPORTS Case Report 1: an 18-Year-Old Track Runner with Exertional Chest Tightness An 18-year-old track runner presents with episodic chest tightness at practices. She notices this sensation most often in the middle of workouts, specifically during the recovery periods between sprint/high-intensity intervals. Her chest tightness is accompanied by the sensation that her heart is beating rapidly and forcefully. She notes some concomitant nonvertiginous lightheadedness but has not had any frank syncope. She is typically unable to resume running until these symptoms terminate, which most often occurs within 10 to 15 min of onset. Her personal and family medical history, physical examination, resting electrocardiogram (ECG), and echocardiogram results are normal. She is taken for exercise testing with the goal of simulating the demands of typical workouts. During customized treadmill exercise testing, she runs 2-min sprint intervals at her usual race pace followed by 1-min recovery intervals. She is asked to continue this sequence until her fatigue becomes prohibitive or she develops her presenting symptoms. After the eighth interval, she begins to experience chest tightness and is noted to have a narrow complex tachycardia at 200 bpm on continuous 12-lead ECG. The morphology is most consistent with a reentrymechanism supraventricular tachycardia. Management options including conservative observation and reassurance, medical therapy with a low-dose negative chronotropic agent, and referral for consideration of an electrophysiology study +/j radiofrequency ablation are discussed. She elects to trial a suppressive medication and is prescribed a low dose of extended-release oral verapamil. She is immediately cleared for returned to practice and is asymptomatic with no perceptible decrement in running performance at 3-, 6-, and 12-month follow-up visits. Key Points: Arrhythmias, specifically supraventricular tachycardias, are an important cause of chest discomfort. Customized exercise testing is often required to reproduce presenting symptoms and underlying pathophysiology. When arrhythmia is suspected and comprehensive laboratory-based exercise testing is unremarkable, extended ambulatory rhythm monitoring is required. Case Report 2: 18-Year-Old Soccer Player with Exertional Chest Pain and Syncope An 18-year-old soccer player presents with recurrent exertional chest pain and one recent episode of syncope. His chest pain symptoms only occur during the middle of his most intense workouts and reach a severity that often causes him to slow down or stop. He describes the discomfort as an ache in his upper chest that is accompanied by mild dyspnea, 250


but no clear wheeze or cough. These symptoms reliably resolve completely after 2 to 3 min of rest. He was recently started empirically on inhaled bronchodilators without improvement in his symptoms. During a recent workout, he had a chest pain episode that culminated in witnessed syncope. Specifically, his teammates and coaching staff noted that he began to clutch his chest while running after a long pass and ultimately fell to ground where he lay motionless for approximately 30 to 45 s. He regained consciousness spontaneously and complained only of transient chest pain. His personal and family medical history, physical examination, and resting ECG results are unremarkable. Transthoracic echocardiography reveals an anomalous left main coronary artery (LMCA) arising from the right coronary sinus. Computerized tomography (CT) coronary angiography confirms this anatomy and notes that the proximal LMCA had a narrow ‘‘slit-like’’ ostium and a proximal course that lead between the aorta and the pulmonary artery. He is removed from athletic participation and referred for cardiac surgery in anticipation of an unroofing procedure. After an uncomplicated cardiac surgical procedure and an 8-wk recovery, he undergoes maximal, effort-limited exercise testing, during which he had no symptoms, ECG changes, or arrhythmia. He was cleared for incremental aerobic training and ball drills. At 4-month follow-up, he feels well and is cleared for return to competitive soccer. Key Points: Exertional chest discomfort that limits effort and/or is accompanied by syncope should prompt immediate restriction from athletic participation until a diagnosis is identified. Syncope that is associated with exertion, rather than immediately following sudden cessation of activity, is of particular concern. Exertional chest discomfort and/or syncope should be considered to be of cardiac origin until Table 1. Cardiac causes of exertional chest pain in athletes.

Coronary artery disorders Atherosclerosis Anomalous origin of coronary artery Coronary artery dissection Coronary artery vasospasm Myocardial bridging Valvular disorders Congenital aortic stenosis Congenital pulmonic stenosis Rheumatic mitral stenosis Mitral valve prolapse Myocardial disorders HCM Myocarditis/pericarditis Aortic disorders Aortic dissection Electrophysiologic disorders Supraventricular arrhythmias

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Table 2. Noncardiac/nonmusculoskeletal medical causes of exertional chest pain.

Diagnosis

Athletes at Most Risk

Return to Competition

History

Diagnosis

Treatment

Asthma

Exertional chest tightness, SOB, cough, or wheezing, starts minutes after exercise or in recovery.

History, examination (wheeze), negative cardiac workup, spirometry with methacholine or exercise challenge

Inhaled Cold environment After negative bronchodilator sports (ice hockey, cardiac workup +/j corticosteroid figure skating), and clinical pool-based improvement athletes, with medical endurance athletes therapy

Pneumothorax

Spontaneous (tall, thin) or after trauma, sharp pain worsening with deep breath

History, examination, AP inspiratory chest film, expiratory film if not seen

Observation versus chest tube

Weightlifting, running, SCUBA. Risk factors: smoking, substance abuse

Following symptomatic and radiographic resolution. Suggested time frame, 4 to 6 wk prior to contact sports

Pulmonary embolus Acute chest pain, new wheeze, pleuritic pain, hemoptysis, and dyspnea

History, examination (tachycardia, tachypnea, leg swelling), chest CT, ventilation perfusion scan

Anticoagulation, fibrinolysis if significant

Athletes at risk for thrombosis (surgery or trauma, fhx of clotting, birth control pills)

Contact sports prohibited while on anticoagulation

Pneumonia

Focal pain, pleuritic or achy in nature; cough, fever

History, examination, PA and lateral chest films

Antibiotics

Athletes with underlying pulmonary issues

After completion of antibiotic course and resolution of symptoms

Pleurisy

Sharp pain History, Nonsteriodal worsening examination anti-inflammatory with deep breath (pleural friction rub) drugs

May occur after respiratory infections, pneumonia, or blunt chest wall trauma ‘‘pulmonary contusions’’

Avoid activities that worsen pain for 2 wk

Runners, jumpers, weightlifters. Risk factors: hiatal hernia, NSAIDs, protein supplements

No restrictions

Pulmonary

GI GERD

Heartburn, chest or epigastric pain, belching, nausea, vomiting

History, usually sufficient, endoscopy or exercise test if needed

Avoid caffeine, alcohol, and hit fat foods. Limit portion sizes during the 4 hours prior to exercise. Trial H2-blockers or proton pump inhibitors.

Musculoskeletal causes of chest pain are detailed in part 3.

proven otherwise. Transient myocardical ischemia due to coronary anomalies with high-risk anatomy is a common and life-threatening cardiac cause of exertional chest discomfort and/or syncope in young athletes. High-risk coronary anomalies, when symptomatic, may present as episodic exertional chest pain and/or syncope. The resting 12-lead ECG is most often normal among patients with coronary anomalies, and www.acsm-csmr.org

exercise stress testing may prove falsely reassuring. If clinical presentation suggests the presence of an underlying coronary anomaly, noninvasive imaging is mandatory to exclude or confirm this diagnosis. Transthoracic echocardiography is the initial imaging test of choice. Additional imaging modalities are required if echocardiography cannot accurately identify normal ostia and proximal course of both coronary arteries Current Sports Medicine Reports


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or to define high-risk features once an anomaly is detected. Athletes found to have an anatomically high-risk coronary anomaly should be restricted until the defect is surgically corrected.

Traumatic rib fracture Traumatic rib fracture is generally caused by blunt trauma to the ribs. Ribs are weakest at the posterior angle; fractures occur most commonly in ribs 4 to 9.

References

Rib stress fractures Rib stress fractures should be considered in any athlete with atraumatic chest wall pain associated with repetitive activity. Magnetic resonance imaging is the imaging modality of choice because of its superior specificity (985%).

1. Baggish AL, Weiner RB, Kanayama G, et al. Long-term anabolic-androgenic steroid use is associated with left ventricular dysfunction. Circ. Heart Fail. 2010; 3:472Y6. 2. Baggish AL, Thompson PD. The Athlete’s Heart 2007: diseases of the coronary circulation. Cardiol. Clin. 2007; 25:431Y40. 3. Kim JH, Malhotra R, Chiampas G, et al. Cardiac arrest during long-distance running races. N. Engl. J. Med. 2012; 366:130Y40. 4. Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA. 1996; 276:199Y204. 5. Perron AD. Chest pain in athletes. Clin. Sports Med. 2003; 22:37Y50.

Chest Pain in Athletes from Personal History Section (Musculoskeletal Causes) Michael J. Khadavi, MD and Michael Fredericson, MD, FACSM 1A. PERSONAL HISTORY: HAVE YOU EVER EXPERIENCED CHEST PAIN OR DISCOMFORT WITH EXERCISE? Chest pain in young athletes is rarely fatal and usually of musculoskeletal origin (2). Once cardiac and other lifethreatening causes of chest pain have been effectively eliminated from the differential diagnosis, musculoskeletal etiologies may be investigated. The following questions may aid in the differentiation of these conditions. Was There Trauma to Your Chest Wall? Chest wall trauma necessitates evaluation for SC joint pathology, slipping rib syndrome, subluxed ribs, or rib fracture. Where Is Your Pain? Determining location of greatest pain followed by careful palpation of the chest wall structures are both useful in narrowing down the differential diagnosis of musculoskeletal chest pain. Is There History of Repetitive Upper Limb or Trunk Use? Overuse phenomena in the upper limbs are common in athletes who repetitively perform a stroke or motion. Evaluate for rib stress fractures, intercostal muscle strain, rib articulation pain, tendon injuries, and costochondritis. Is Your Pain Worse with Exercise? Pain with specific upper limb movements may occur with a rib stress fracture, rib subluxations, or intercostal strain. On the other hand, conditions that are not exacerbated by exercise include precordial catch syndrome, Tietze syndrome, and cervical etiologies of chest pain. A differential diagnosis of musculoskeletal chest pain should include the following common and clinically significant etiologies. 252


Sternoclavicular injury Sternoclavicular (SC) joint injuries may occur in collision sports. These injuries may be a source of chronic pain in some athletes. The physical examination will demonstrate pain on palpation directly over the SC joint. Exercise-related transient abdominal pain ("side stitch") This is a benign condition that athletes describe as a sharp, crampy, or pleuritic pain in the abdomen or lower chest. It occurs only with exercise and disappears with rest. While the etiology is not completely understood, it is often experienced by less trained individuals during vigorous, prolonged exercise or repetitive torso movement, particularly when performed within an hour of eating or drinking. Costochondritis Costochondritis presents as a peristernal sharp or pressurelike pain, most commonly at second through fifth sternocostal articulations. Athletes may complain of unilateral symptoms that are worse with deep inspiration and upper limb movements after activities that involve repetitive, strenuous use of the chest musculature such as rowing or weightlifting. Diagnosis is made by reproducing the symptoms with palpation of the affected area and exclusion of other potential causes. Intercostal muscle injury Pain between the ribs exacerbated by movement, coughing, or deep inspiration may indicate a sprain of the intercostal or other chest wall musculature. The athlete will often recall a specific a sudden increase in training or a specific incident during activity when the pain began. Slipping rib syndrome Intermittent sharp pain in the lower ribcage is typical of slipping rib syndrome. This pain may radiate into the chest or back, and the athlete may describe a ‘‘popping’’ or ‘‘slipping’’ sensation of the ribs. Certain postures, trunk movements, and deep breathing may precipitate these symptoms, and this diagnosis is most common in sports with frequent arm abduction. The ‘‘hooking maneuver,’’ performed by placing fingers beneath the lower costal margin and pulling upward and outward is positive if it reproduces the athlete’s pain or a click due to hypermobility of the lower ribs. Precordial catch syndrome Precordial catch syndrome is characterized by sharp, stabbing pain in the left precordial and parasternal region that lasts a few seconds; this syndrome accounts for approximately 15% of noncardiac chest pain cases and is unique because it may occur at rest or with mild activity (2). Cardiovascular Preparticipation Evaluation


or to define high-risk features once an anomaly is detected. Athletes found to have an anatomically high-risk coronary anomaly should be restricted until the defect is surgically corrected.

Traumatic rib fracture Traumatic rib fracture is generally caused by blunt trauma to the ribs. Ribs are weakest at the posterior angle; fractures occur most commonly in ribs 4 to 9.

References

Rib stress fractures Rib stress fractures should be considered in any athlete with atraumatic chest wall pain associated with repetitive activity. Magnetic resonance imaging is the imaging modality of choice because of its superior specificity (985%).

1. Baggish AL, Weiner RB, Kanayama G, et al. Long-term anabolic-androgenic steroid use is associated with left ventricular dysfunction. Circ. Heart Fail. 2010; 3:472Y6. 2. Baggish AL, Thompson PD. The Athlete’s Heart 2007: diseases of the coronary circulation. Cardiol. Clin. 2007; 25:431Y40. 3. Kim JH, Malhotra R, Chiampas G, et al. Cardiac arrest during long-distance running races. N. Engl. J. Med. 2012; 366:130Y40. 4. Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA. 1996; 276:199Y204. 5. Perron AD. Chest pain in athletes. Clin. Sports Med. 2003; 22:37Y50.

Chest Pain in Athletes from Personal History Section (Musculoskeletal Causes) Michael J. Khadavi, MD and Michael Fredericson, MD, FACSM 1A. PERSONAL HISTORY: HAVE YOU EVER EXPERIENCED CHEST PAIN OR DISCOMFORT WITH EXERCISE? Chest pain in young athletes is rarely fatal and usually of musculoskeletal origin (2). Once cardiac and other lifethreatening causes of chest pain have been effectively eliminated from the differential diagnosis, musculoskeletal etiologies may be investigated. The following questions may aid in the differentiation of these conditions. Was There Trauma to Your Chest Wall? Chest wall trauma necessitates evaluation for SC joint pathology, slipping rib syndrome, subluxed ribs, or rib fracture. Where Is Your Pain? Determining location of greatest pain followed by careful palpation of the chest wall structures are both useful in narrowing down the differential diagnosis of musculoskeletal chest pain. Is There History of Repetitive Upper Limb or Trunk Use? Overuse phenomena in the upper limbs are common in athletes who repetitively perform a stroke or motion. Evaluate for rib stress fractures, intercostal muscle strain, rib articulation pain, tendon injuries, and costochondritis. Is Your Pain Worse with Exercise? Pain with specific upper limb movements may occur with a rib stress fracture, rib subluxations, or intercostal strain. On the other hand, conditions that are not exacerbated by exercise include precordial catch syndrome, Tietze syndrome, and cervical etiologies of chest pain. A differential diagnosis of musculoskeletal chest pain should include the following common and clinically significant etiologies. 252


Sternoclavicular injury Sternoclavicular (SC) joint injuries may occur in collision sports. These injuries may be a source of chronic pain in some athletes. The physical examination will demonstrate pain on palpation directly over the SC joint. Exercise-related transient abdominal pain ("side stitch") This is a benign condition that athletes describe as a sharp, crampy, or pleuritic pain in the abdomen or lower chest. It occurs only with exercise and disappears with rest. While the etiology is not completely understood, it is often experienced by less trained individuals during vigorous, prolonged exercise or repetitive torso movement, particularly when performed within an hour of eating or drinking. Costochondritis Costochondritis presents as a peristernal sharp or pressurelike pain, most commonly at second through fifth sternocostal articulations. Athletes may complain of unilateral symptoms that are worse with deep inspiration and upper limb movements after activities that involve repetitive, strenuous use of the chest musculature such as rowing or weightlifting. Diagnosis is made by reproducing the symptoms with palpation of the affected area and exclusion of other potential causes. Intercostal muscle injury Pain between the ribs exacerbated by movement, coughing, or deep inspiration may indicate a sprain of the intercostal or other chest wall musculature. The athlete will often recall a specific a sudden increase in training or a specific incident during activity when the pain began. Slipping rib syndrome Intermittent sharp pain in the lower ribcage is typical of slipping rib syndrome. This pain may radiate into the chest or back, and the athlete may describe a ‘‘popping’’ or ‘‘slipping’’ sensation of the ribs. Certain postures, trunk movements, and deep breathing may precipitate these symptoms, and this diagnosis is most common in sports with frequent arm abduction. The ‘‘hooking maneuver,’’ performed by placing fingers beneath the lower costal margin and pulling upward and outward is positive if it reproduces the athlete’s pain or a click due to hypermobility of the lower ribs. Precordial catch syndrome Precordial catch syndrome is characterized by sharp, stabbing pain in the left precordial and parasternal region that lasts a few seconds; this syndrome accounts for approximately 15% of noncardiac chest pain cases and is unique because it may occur at rest or with mild activity (2). Cardiovascular Preparticipation Evaluation


This benign condition of unknown etiology is often exacerbated by deep inspiration and is associated with slouched position that resolves with progression into an upright posture. Reassurance is the only management necessary.

Table 2.

Pain with Exertion

Pain Commonly at Rest

Cervical disk disease/"cervical angina" Anterior or posterior chest pain may be caused by disorders of the cervical spine, such as cervical disk disease, ossification of the posterior longitudinal ligament, or a C6 or C7 radiculopathy. The history examination should inquire about alteration of the pain with cervical movements, and physical examination should include cervical range of motion, the Spurling maneuver, and a neurologic assessment of the upper and lower limbs with careful documentation of deficits.

& Rib fracture

& Precordial catch syndrome

Tietze syndrome Acute, unilateral, anterior chest pain is the usual presentation of this benign condition, distinguished from costochondritis by the presence of swelling at the sternocostal articulation. Subluxed rib at the costovertebral (rib head) or costochondral joints/articulations Joint articulation is ‘‘stuck’’ out of normal position and causes pain with joint movement and reactive muscle spasm. The pain can be located anywhere along the rib distribution. There is usually tenderness at the rib head junction. This can be treated with manual therapy and may self-reduce with time. Case Report 1: Rib Stress Fracture A 19-year-old female rower presented with a 2-wk history of insidious onset of left lateral and anterior chest pain. There was no pain with moderate exercise such as walking, elliptical, or light running, but the pain occurred when running at a faster pace and rowing. She reported that during the offseason, she pursued a high-resistance, low-repetition rowing routine. Cardiac examination result was normal. There was focal tenderness over the left eighth rib laterally and no tenderness over the pectoralis, latissimus dorsi, or trapezius. Sidebending and deep inspiration reproduced her pain. Rib-view Table 1. Association with chest wall trauma and overuse in musculoskeletal chest wall pain.

History of Trauma to Chest Wall

History of Repetitive Use of Trunk or Upper Limbs

No History of Chest Wall Trauma or Overuse

& Rib fracture

& Rib stress fracture & Side stitch’’

& SC injury

& Costochondritis

& Precordial catch syndrome

& Slipping rib syndrome & Intercostals muscle & ‘‘Cervical (common, not injury angina’’ necessary for diagnosis) & Tietze syndrome (rarely) & Subluxed rib

www.acsm-csmr.org

& Subluxed rib

& Tietze syndrome

Musculoskeletal etiologies of chest pain often display patterns in their presentation, based on their association with rest or exertion.

& Rib stress fracture

& Tietze syndrome

& SC injury

& Cervical angina

& ‘‘Side stitch’’ & Costochondritis & Intercostal strain & Slipping rib syndrome & Subluxed rib

plain radiographs were negative for osseous abnormality. The patient was treated conservatively for a stress fracture of her eighth rib, including reduction in activity of the upper limbs, cross-training, calcium and vitamin D supplementation, and acetaminophen for pain control. After 1 wk of rest, she was able to progress to upper limb exercises pain-free and returned to full activity within 3 wk with the help of a focused physical therapy program. Key Point: Insidious onset of chest wall pain in an individual with repetitive upper limb use necessitates the inclusion of a rib stress fracture in the differential diagnosis. Case Report 2: Precordial Cath Syndrome A 16-year-old female student athlete on her high school basketball team presented to the sports medicine clinic for leftsided chest pain and trouble breathing. During practice the previous day, she reported these symptoms to her coach. Her coach sent her to the emergency room, and chest x-ray and electrocardiogram results were normal. She described a sharp, stabbing pain in the left parasternal chest that only lasted a few seconds while warming up in practice. When this happened, she had trouble fully inhaling, and after several seconds of shallow breathing, the sharp pain resolved and was followed by a dull ache for about 5 min. She reported a similar episode this morning in class while she was sitting in a slouched position that resolved when she stretched her trunk into a more erect, upright position. A complete musculoskeletal examination of the upper limbs and chest wall yielded negative results, and she was diagnosed with precordial catch syndrome and counseled regarding this common, benign condition that is managed conservatively with postural changes as she performed this morning in class. Key Point: Precordial catch syndrome is a benign condition characterized by sharp pain of short duration that is often influenced by trunk position or respiration. Case Report 3: Intercostal Muscle Strain A 23-year-old left-handed male reported right lateral chestwall pain since he participated in a ‘‘long drive’’ competition for golfers 5 d ago. He attempted to play again 2 d ago but quit early because of this pain. He denied dyspnea, radiation of his pain, or pain during cardiovascular exercise but noticed Current Sports Medicine Reports


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that his chest hurt yesterday during a coughing spell. Upon physical examination, there was mild tenderness in the area of the right sixth through eighth ribs laterally that was more pronounced in the intercostal segments than over the ribs themselves. Pain also was reproduced with side-bending to the left. After negative plain radiographs, he was diagnosed with strained intercostal muscles, a common phenomenon after excessive muscular activity of the upper limbs and trunk. He was given a 2-wk course of nonsteroidal anti-inflammatory drugs, counseled to refrain from swinging for at least a week, and prescribed a course of physical therapy. Within 3 wk, he was back to full, pain-free activities. Key Point: Intercostal muscle strain symptoms are reproduced with forceful contraction, passive stretching, and palpation and improve with rest and conservative care. References 1. Gregory PL, Biswas AC, Batt ME. Musculoskeletal problems of the chest wall in athletes. Sports Med. 2002; 32:235Y50. 2. Saleeb SF, Li WY, Warren SZ, Lock JE. Effectiveness of screening for lifethreatening chest pain in children. Pediatrics. 2011; 128:1062Y8. 3. Sert A, Aypar E, Odabas D, Gokcen C. Clinical characteristics and causes of chest pain in 380 children referred to a paediatric cardiology unit. Cardiol. Young. 2013; 23:361Y7.

Syncope in Athletes of Cardiac Origin: 2B. From Personal History and Physical Examination Sections Francis G. O’Connor, MD, MPH, FACSM and Benjamin Levine, MD Personal History: Have You Ever Nearly Lost or Actualy Lost Consciousness? The History To determine the importance of a positive answer to this question, the following questions should be asked:

1. Did the syncopal event occur during or immediately after exercise; if the latter, was the patient standing still or moving around? 2. Prior to the syncopal event, were there any prodromal symptoms such as the following: chest pain, palpitations, visual changes, wheezing/shortness of breath, nausea, or itching? 3. After the syncopal event, what was the patient’s postevent clinical status? Did they wake quickly, or was there a prolonged period of unresponsiveness? Was there any observed seizure activity or loss of urine, stool, or tongue bite marks? Were there vital signs recorded on site, particularly the immediate postsyncope measurements? 4. What medications and supplements were being utilized prior to the event? 5. Is there a prior personal or family history of syncope or of sudden cardiac arrest/death? The evaluating physician’s first priority is to use the clinical history to distinguish between true syncope involving a loss of 254


consciousness with presumed hemodynamic compromise and exercise-associated collapse associated with exhaustive effort or postexercise hypotension. In true syncope from hemodynamic causes, the athlete typically recovers quickly with restoration of arterial pressure, unless resuscitation is required. After collapse associated with an exhaustive effort, however, athletes usually will have prolonged periods of ‘‘being out of it,’’ even in the supine position with normal heart rate and BP. This picture is in contrast to patients with syncope due to heat stress who are universally hypotensive and tachycardic. Athletes who are impaired, seeming ‘‘unconscious,’’ but able to assist in their own evacuation are unlikely to be in the throes of a life-threatening arrhythmia, although other metabolic or catastrophic abnormalities are possible (e.g., hyponatremia, exertional heat stroke). The postevent state provides important clues such as seizure activity, incontinence, and immediate vital signs (including body temperature). It must be emphasized however, that a seizure-like activity can be the result of reduced cerebral perfusion and therefore does not always imply epilepsy or neurologic problems. Reports from witnesses can be invaluable. Too often, syncope is assumed to be neurologic and the cardiac evaluation is not done, resulting in fatal outcomes. The second critical distinction is whether the event occurred during or immediately after exercise. Orthostatic hypotension occurring after exercise, usually associated with sudden cessation of activity, is much less ominous than the sudden loss of consciousness that occurs during exercise, which suggests cardiac arrhythmia with loss of blood flow to the brain. Syncope prompted by an abrupt loud noise such as a starting gun or immersion in cold water may provide a clinical clue to prolonged QT syndrome (LQTS). The third component of the history that provides clinical clues is a detailed assessment of events prior to the collapse. Prodromal symptoms such as palpitations (suggesting arrhythmia), chest pain (ischemia, aortic dissection), nausea (ischemia, high levels of vagal activity, or hyponatremia), wheezing, and pruritus (anaphylaxis) are significant, along with precipitating events like ‘‘only during exercise.’’ It is important to identify whether syncope occurs in the upright position (orthostatic hypotension) alone or also when sitting or supine (arrhythmia or nonhemodynamic cause). The fourth component of the evaluation requires assessment of the medication and supplement history. A comprehensive medication list, including over-the-counter medications and ergogenic aids is necessary; the practice of high-risk behaviors, such as recreational drug use should be carefully investigated. Finally, a personal and family history of sudden death is critical and, if present, may identify very high-risk subgroups with hypertrophic cardiomyopathy (HCM), LQTS, or right ventricular cardiomyopathy. Delineating a family tree will give a complete snapshot of the family history.

The Physical Examination Vital signs (including orthostatic measurements) should be included. Blood pressure (BP) should be measured in both arms and legs as well as after 5 min of moving to a standing position. Cardiovascular Preparticipation Evaluation


that his chest hurt yesterday during a coughing spell. Upon physical examination, there was mild tenderness in the area of the right sixth through eighth ribs laterally that was more pronounced in the intercostal segments than over the ribs themselves. Pain also was reproduced with side-bending to the left. After negative plain radiographs, he was diagnosed with strained intercostal muscles, a common phenomenon after excessive muscular activity of the upper limbs and trunk. He was given a 2-wk course of nonsteroidal anti-inflammatory drugs, counseled to refrain from swinging for at least a week, and prescribed a course of physical therapy. Within 3 wk, he was back to full, pain-free activities. Key Point: Intercostal muscle strain symptoms are reproduced with forceful contraction, passive stretching, and palpation and improve with rest and conservative care. References 1. Gregory PL, Biswas AC, Batt ME. Musculoskeletal problems of the chest wall in athletes. Sports Med. 2002; 32:235Y50. 2. Saleeb SF, Li WY, Warren SZ, Lock JE. Effectiveness of screening for lifethreatening chest pain in children. Pediatrics. 2011; 128:1062Y8. 3. Sert A, Aypar E, Odabas D, Gokcen C. Clinical characteristics and causes of chest pain in 380 children referred to a paediatric cardiology unit. Cardiol. Young. 2013; 23:361Y7.

Syncope in Athletes of Cardiac Origin: 2B. From Personal History and Physical Examination Sections Francis G. O’Connor, MD, MPH, FACSM and Benjamin Levine, MD Personal History: Have You Ever Nearly Lost or Actualy Lost Consciousness? The History To determine the importance of a positive answer to this question, the following questions should be asked:

1. Did the syncopal event occur during or immediately after exercise; if the latter, was the patient standing still or moving around? 2. Prior to the syncopal event, were there any prodromal symptoms such as the following: chest pain, palpitations, visual changes, wheezing/shortness of breath, nausea, or itching? 3. After the syncopal event, what was the patient’s postevent clinical status? Did they wake quickly, or was there a prolonged period of unresponsiveness? Was there any observed seizure activity or loss of urine, stool, or tongue bite marks? Were there vital signs recorded on site, particularly the immediate postsyncope measurements? 4. What medications and supplements were being utilized prior to the event? 5. Is there a prior personal or family history of syncope or of sudden cardiac arrest/death? The evaluating physician’s first priority is to use the clinical history to distinguish between true syncope involving a loss of 254


consciousness with presumed hemodynamic compromise and exercise-associated collapse associated with exhaustive effort or postexercise hypotension. In true syncope from hemodynamic causes, the athlete typically recovers quickly with restoration of arterial pressure, unless resuscitation is required. After collapse associated with an exhaustive effort, however, athletes usually will have prolonged periods of ‘‘being out of it,’’ even in the supine position with normal heart rate and BP. This picture is in contrast to patients with syncope due to heat stress who are universally hypotensive and tachycardic. Athletes who are impaired, seeming ‘‘unconscious,’’ but able to assist in their own evacuation are unlikely to be in the throes of a life-threatening arrhythmia, although other metabolic or catastrophic abnormalities are possible (e.g., hyponatremia, exertional heat stroke). The postevent state provides important clues such as seizure activity, incontinence, and immediate vital signs (including body temperature). It must be emphasized however, that a seizure-like activity can be the result of reduced cerebral perfusion and therefore does not always imply epilepsy or neurologic problems. Reports from witnesses can be invaluable. Too often, syncope is assumed to be neurologic and the cardiac evaluation is not done, resulting in fatal outcomes. The second critical distinction is whether the event occurred during or immediately after exercise. Orthostatic hypotension occurring after exercise, usually associated with sudden cessation of activity, is much less ominous than the sudden loss of consciousness that occurs during exercise, which suggests cardiac arrhythmia with loss of blood flow to the brain. Syncope prompted by an abrupt loud noise such as a starting gun or immersion in cold water may provide a clinical clue to prolonged QT syndrome (LQTS). The third component of the history that provides clinical clues is a detailed assessment of events prior to the collapse. Prodromal symptoms such as palpitations (suggesting arrhythmia), chest pain (ischemia, aortic dissection), nausea (ischemia, high levels of vagal activity, or hyponatremia), wheezing, and pruritus (anaphylaxis) are significant, along with precipitating events like ‘‘only during exercise.’’ It is important to identify whether syncope occurs in the upright position (orthostatic hypotension) alone or also when sitting or supine (arrhythmia or nonhemodynamic cause). The fourth component of the evaluation requires assessment of the medication and supplement history. A comprehensive medication list, including over-the-counter medications and ergogenic aids is necessary; the practice of high-risk behaviors, such as recreational drug use should be carefully investigated. Finally, a personal and family history of sudden death is critical and, if present, may identify very high-risk subgroups with hypertrophic cardiomyopathy (HCM), LQTS, or right ventricular cardiomyopathy. Delineating a family tree will give a complete snapshot of the family history.

The Physical Examination Vital signs (including orthostatic measurements) should be included. Blood pressure (BP) should be measured in both arms and legs as well as after 5 min of moving to a standing position. Cardiovascular Preparticipation Evaluation


Special Tests/Referral The three tests that are core to the diagnostic evaluation of the athlete with syncope include the electrocardiogram (ECG), the echocardiogram, and the exercise stress test. Echocardiography should precede the exercise test, allowing the assessment of ventricular size and function, pulmonary pressures, and valve function/integrity. Rather than a standard Bruce protocol, a maximal symptom-limited exercise test should be used to reproduce the conditions that provoked the syncopal event. For example, a stuttering startstop test for a basketball or soccer player or a prolonged high-intensity race pace test for a runner. The exercise ECG also should be examined for appropriate shortening of the QT interval. Further testing should be ordered only as indicated and in consultation with the appropriate specialist. A complete review of all the advanced diagnostic tests is beyond the focus of this article. Tilt table testing should not be used in the syncope evaluation because of the high rate of false positive tests due to the steep Starling curve in athletes. The clinician is additionally reminded that not all syncope is cardiogenic. Athletes whose history suggests seizure activity may require an electroencephalogram and magnetic resonance imaging (MRI) of the brain to exclude a structural lesion, and seizures can be related to hyponatremia or heat stroke. Hematologic and metabolic abnormalities require testing as indicated, e.g., hypoglycemia in those with diabetes, athletes with eating disorders, or patients on beta-blockers. CASE EXAMPLES Case 1: Cross-Country Runner with Collapse at the Finish Line A 17-year-old female track star was competing in the district championships in the 5,000 m. She was having an excellent race and was part of the final pack that was in contention for a medal. Just after crossing the finish line (in 17 min 55 s), she collapsed to the ground and, according to bystanders, remained ‘‘unconscious’’ for about an hour. ECG, echocardiogram, and Holter monitor testing results were normal, although she did not faint during the period of recording. An athlete-specific exercise test was performed on a large treadmill at 10 mph; after 10 min, the patient started to cry and then slumped in the safety harness. At the moment of fainting, her rhythm was normal, pulse was 185 bpm, and BP was 140/70. The final impression was exercise-associated collapse, and the recommendation was better training and pacing to match demands of her sport. Key Point: History pointed away from life-threatening arrhythmia; athlete-specific exercise testing reproduced the problem during direct electrical and hemodynamic monitoring, allowing an accurate diagnosis. Case 2: Basketball Player with Collapse on the Free Throw Line A 21-year-old male college basketball star led a 12-point run, mostly by a series of impressive fast breaks, in the middle of the first half of an early round of the National Collegiate Athletic Association tournament. After the last www.acsm-csmr.org

jam, he was fouled and walked to the foul line. While standing waiting to shoot, he slumped to the ground. Witnesses observed generalized seizure activity. ECG showed LVH and unusual T waves in the right precordial leads, but his echocardiogram result was normal and short-term Holter monitoring did not identify any arrhythmias, although no symptoms occurred during the monitoring period. A stuttering exercise test was performed at high speed (15 mph) followed by sudden stops. After the fifth repeat, while standing quietly, there was a sudden drop in pulse to 45 bpm, BP was 60/40, and the patient fainted on the treadmill. The final diagnosis was postexercise hypotension (neurally mediated syncope). The recommendation was behavioral modification (avoid standing still if possible, learn leg crossing/butt clenching and toe bouncing maneuvers, stay well hydrated, and wear compression socks). Key Point: Standing still after intense exercise can lead to hypotension due to loss of the muscle pump and lead to neurally mediated syncope with bradycardia and hypotension. Athlete-specific exercise testing elicited the problem and led to simple, effective, nonpharmacologic therapy. Case 3: Football Player with Collapse while Running out for a Pass A 19-year-old male wide receiver for a junior college football team was running a crossing pattern during practice when witnesses observed him to weave, stumble, and fall to the ground. The trainer arrived at the scene to find him alert and oriented. His BP was 135/80; pulse was 110 bpm and regular. Physical examination result was normal; however, ECG results showed septal Q waves, LVH, and deep T wave inversions. Echocardiogram results showed a septal thickness of 1.8 cm, although without systolic anterior motion of the mitral valve. Holter monitoring revealed multiple runs of NSVT, and cardiac MRI showed multiple areas of delayed enhancement. The diagnosis was HCM; he was held from competition, and family members were screened for HCM. Key Point: Syncope during exercise is very concerning for an arrhythmia and underlying structural heart disease. Case 4: Lacrosse Player with Syncope during Exercise A lacrosse player had syncope while running wind sprints. Careful history examination documented that the syncope occurred during a sprint and not between sprints. ECG results showed nonspecific ECG changes, but the echocardiogram result was normal. The exercise test result was normal; cardiac MRI was performed and showed patchy areas of delayed enhancement indicating potential myocarditis. An electrophysiology (EP) study induced atrioventricular nodal reentrant tachycardia (AVNRT) but no ventricular arrhythmias. A 24-h Holter monitor showed no arrhythmias. The athlete had a major game coming up, and there was pressure to allow him to play. The patient had ablation of the AVNRT and was allowed to return to sports. He died in practice the following week. Key Point: The worrisome history plus noncoronary distribution of delayed enhancement on MRI are concerning for myocarditis. The conservative approach would have been Current Sports Medicine Reports


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to treat with a beta-blocker and angiotensin-converting enzyme inhibitor, hold out of sports for at least 3 to 6 months, perhaps implant an implantable loop recorder so that a specific diagnosis could be made if another episode of syncope occurred, and then repeat the MRI looking for resolution of delayed enhancement. Pressure to play (athlete, coach, and parent) can influence medical decision making and steer the decision away from the athlete’s best interest. Case 5: A 23-year-old male second-year medical student completed a maximal-effort Wingate test as part of a research project; prior V˙O2max testing demonstrated a maximum of 55 mLIkgj1Iminj1. Immediately after the 30-s test, the student was extremely nauseous and rapidly exited the bike to move to a trash can to vomit; after two short wobbly steps, he collapsed to the ground. He was attended to immediately by the exercise staff in attendance; he had an estimated loss of consciousness for less than 30 s and had a first set of vital signs of heart rate of 140 bpm and BP of 110/60. There was no observed seizure activity and no prior complaint of chest pain. A subsequent evaluation in the sports medicine clinic demonstrated normal clinical examination results with no history of syncope; ECG results demonstrated early repolarization variation in the precordial leads. As the syncopal event occurred immediately after exercise, a treadmill stress test and echocardiogram were performed; the student completed 17 min of Bruce protocol and had a normal echocardiogram results. The diagnosis was postexertional syncope secondary to orthostatic hypotension. Key Point: Syncope during or immediately after exercise is always concerning and warrants conscientious examination to rule out structural cardiovascular disease. Intense anaerobic exercise, which can be seen in maximal efforts like the Wingate test or weightlifting, can precipitate syncope through orthostatic hypotension and a central effect of hypocapnia leading to, or aggravating, cerebral hypoperfusion. The history of the event provides not only insight into the diagnosis but also important opportunities for prudent recommendations for prevention.

Syncope in Athletes of Neurological Origin: 2B. From Personal History and Physical Examination Sections Chad A. Asplund, MD, MPH, FACSM and Jeffrey S. Kutcher, MD Personal History: Have You Ever Nearly Lost or Actually Lost Consciousness? The History (Regarding Neurologic Causes) To determine the importance of a positive answer to this question, the following questions should be asked for each episode of loss of consciousness:

1. Was consciousness completely lost? If so, for how long? 2. Was head trauma involved? 256


3. Was the episode witnessed? What did observers say happened? 4. Was a convulsion involved? If so, what was the duration of the convulsion and how long was the postictal period? 5. Was the episode preceded by fear, pain, prolonged standing, psychologic stress, or a medical procedure? 6. Was the episode preceded by a headache or visual symptoms? 7. Was the episode preceded by heart palpitations, lightheadedness, or tunnel vision? 8. Was the episode concurrent with the use of medications, performance enhancing substances, or illicit drugs? The first priority in the evaluation of a patient with history of loss of consciousness begins with thorough and directed history examination. It is imperative that the evaluator differentiate true syncope with transient loss of consciousness from presyncope, where consciousness is not lost. Presyncope is rarely caused by neurologic disease alone and may be a manifestation of cardiac disease, anxiety, or the side effect of a medication or supplement. If there is any concern for cardiac etiology, then at a minimum, an electrocardiogram should be performed before the neurologic evaluation continues. The second priority of the history examination is to focus on circumstances immediately before the event, the onset, the event itself, and the postevent period. Various aspects surrounding the event may help establish the diagnosis. A prodrome, postsyncopal fatigue, amnesia, or focal neurologic deficits will point the evaluator toward a neurogenic cause. Episodes of neurally mediated syncope are typically associated with postepisode fatigue and weakness, whereas the absence of a prodrome is more consistent with cardiac arrhythmia. Sensory auras, de´ja` vu or jamais vu, postictal confusion, or focal neurologic signs or symptoms all suggest a neurologic cause. Head trauma preceding loss of consciousness is highly suggestive of brain injury, such as concussion, cerebral contusion, or intracranial hemorrhage. Next, the observations of witnesses may be extremely helpful. Preevent behaviors, such as unresponsiveness or automatisms, are suggestive of seizure, as is tonic-clonic, convulsive activity. It is important to recognize that seizurelike activity that occurs prior to loss of consciousness or postural tone is more consistent with seizure while a convulsion that occurs after the patient has fallen to the ground may be the final common manifestation of cerebral hypoperfusion. Incontinence of bowel or bladder does not reliably differentiate neurologic from cardiac causes. Finally, medical history can be very helpful. History of psychologic or psychiatric disorders may point to a nonorganic cause of syncope such as a conversion disorder. History of migraine headaches should be considered as another possible cause for neurogenic syncope. In most patients, the cause of syncope can be determined with great accuracy from careful history and physical examination. However, the exact mechanism of syncope remains unexplained in approximately 35% of episodes and a neurologic cause for syncope is found in fewer than 10% of cases (1). Cardiovascular Preparticipation Evaluation


to treat with a beta-blocker and angiotensin-converting enzyme inhibitor, hold out of sports for at least 3 to 6 months, perhaps implant an implantable loop recorder so that a specific diagnosis could be made if another episode of syncope occurred, and then repeat the MRI looking for resolution of delayed enhancement. Pressure to play (athlete, coach, and parent) can influence medical decision making and steer the decision away from the athlete’s best interest. Case 5: A 23-year-old male second-year medical student completed a maximal-effort Wingate test as part of a research project; prior V˙O2max testing demonstrated a maximum of 55 mLIkgj1Iminj1. Immediately after the 30-s test, the student was extremely nauseous and rapidly exited the bike to move to a trash can to vomit; after two short wobbly steps, he collapsed to the ground. He was attended to immediately by the exercise staff in attendance; he had an estimated loss of consciousness for less than 30 s and had a first set of vital signs of heart rate of 140 bpm and BP of 110/60. There was no observed seizure activity and no prior complaint of chest pain. A subsequent evaluation in the sports medicine clinic demonstrated normal clinical examination results with no history of syncope; ECG results demonstrated early repolarization variation in the precordial leads. As the syncopal event occurred immediately after exercise, a treadmill stress test and echocardiogram were performed; the student completed 17 min of Bruce protocol and had a normal echocardiogram results. The diagnosis was postexertional syncope secondary to orthostatic hypotension. Key Point: Syncope during or immediately after exercise is always concerning and warrants conscientious examination to rule out structural cardiovascular disease. Intense anaerobic exercise, which can be seen in maximal efforts like the Wingate test or weightlifting, can precipitate syncope through orthostatic hypotension and a central effect of hypocapnia leading to, or aggravating, cerebral hypoperfusion. The history of the event provides not only insight into the diagnosis but also important opportunities for prudent recommendations for prevention.

Syncope in Athletes of Neurological Origin: 2B. From Personal History and Physical Examination Sections Chad A. Asplund, MD, MPH, FACSM and Jeffrey S. Kutcher, MD Personal History: Have You Ever Nearly Lost or Actually Lost Consciousness? The History (Regarding Neurologic Causes) To determine the importance of a positive answer to this question, the following questions should be asked for each episode of loss of consciousness:

1. Was consciousness completely lost? If so, for how long? 2. Was head trauma involved? 256


3. Was the episode witnessed? What did observers say happened? 4. Was a convulsion involved? If so, what was the duration of the convulsion and how long was the postictal period? 5. Was the episode preceded by fear, pain, prolonged standing, psychologic stress, or a medical procedure? 6. Was the episode preceded by a headache or visual symptoms? 7. Was the episode preceded by heart palpitations, lightheadedness, or tunnel vision? 8. Was the episode concurrent with the use of medications, performance enhancing substances, or illicit drugs? The first priority in the evaluation of a patient with history of loss of consciousness begins with thorough and directed history examination. It is imperative that the evaluator differentiate true syncope with transient loss of consciousness from presyncope, where consciousness is not lost. Presyncope is rarely caused by neurologic disease alone and may be a manifestation of cardiac disease, anxiety, or the side effect of a medication or supplement. If there is any concern for cardiac etiology, then at a minimum, an electrocardiogram should be performed before the neurologic evaluation continues. The second priority of the history examination is to focus on circumstances immediately before the event, the onset, the event itself, and the postevent period. Various aspects surrounding the event may help establish the diagnosis. A prodrome, postsyncopal fatigue, amnesia, or focal neurologic deficits will point the evaluator toward a neurogenic cause. Episodes of neurally mediated syncope are typically associated with postepisode fatigue and weakness, whereas the absence of a prodrome is more consistent with cardiac arrhythmia. Sensory auras, de´ja` vu or jamais vu, postictal confusion, or focal neurologic signs or symptoms all suggest a neurologic cause. Head trauma preceding loss of consciousness is highly suggestive of brain injury, such as concussion, cerebral contusion, or intracranial hemorrhage. Next, the observations of witnesses may be extremely helpful. Preevent behaviors, such as unresponsiveness or automatisms, are suggestive of seizure, as is tonic-clonic, convulsive activity. It is important to recognize that seizurelike activity that occurs prior to loss of consciousness or postural tone is more consistent with seizure while a convulsion that occurs after the patient has fallen to the ground may be the final common manifestation of cerebral hypoperfusion. Incontinence of bowel or bladder does not reliably differentiate neurologic from cardiac causes. Finally, medical history can be very helpful. History of psychologic or psychiatric disorders may point to a nonorganic cause of syncope such as a conversion disorder. History of migraine headaches should be considered as another possible cause for neurogenic syncope. In most patients, the cause of syncope can be determined with great accuracy from careful history and physical examination. However, the exact mechanism of syncope remains unexplained in approximately 35% of episodes and a neurologic cause for syncope is found in fewer than 10% of cases (1). Cardiovascular Preparticipation Evaluation


The Physical Examination A complete neurologic examination should be performed following a suspected neurogenic cause of syncope. Abnormalities of cognition and speech, visual fields, motor strength, sensation, tremor, and gait suggest an underlying neurologic disorder. The Physical Examination If an underlying neurologic disorder is suspected, further study and subspecialty consultation should be considered. If the event was preceded by head trauma with subsequent neurologic deficits, a computed tomography (CT) scan of the head to assess for gross intracranial pathology should be considered, but magnetic resonance imaging (MRI) will yield better resolution for structural or brain tissue pathology. Finally, if seizure activity is suspected, an electroencephalogram (EEG) may be warranted. If the event was preceded by fear, pain, prolonged standing, psychologic stress, or a medical procedure and the athlete has normal physical examination results, the diagnosis of vasovagal syncope or neurally mediated syncope can be made. CASE REPORTS Case 1: Cross-Country Runner with Loss of Consciousness while Running A 15-year-old male cross country runner reported loss of consciousness with exercise during his PPE. Since he was uncertain of the details, his coaches were questioned. They said he stopped running shortly after beginning the second of a two-lap course, had a contusion and laceration to the forehead, and was confused. He denies recall of what happened after he slipped, but the coaches stated that he had fallen, struck his head on a rock, and appeared to have lost consciousness for several seconds. They denied seeing any seizure-like activity. Key Point: Concussions can occur in sports other than traditional ‘‘contact/collision’’ sports and are a common cause of loss of consciousness in athletes. Case 2: Track Athlete with Loss of Consciousness during a Race A 21-year-old female track runner reported loss of consciousness during a 200-m race during her PPE. Her coaches said she was prone and unresponsive on the track, with tremulous activity in both upper and lower extremities lasting about 90 s, after which she was responsive and aware of her situation. She reported having three similar episodes while in high school. There were no other significant personal or family medical history, medication, or supplement use. Neurologic examination result was normal except for the transient inability to move her left arm. Brain imaging (CT and MRI) results were normal. When questioned about stress, she stated she had a previous injury, which occurred in the middle of a championship 200-m race, and she worried constantly about being injured again and unable to compete. Following 48 h of cognitive behavioral therapy, she improved and was able to move all extremities. She was diagnosed with conversion disorder as the cause of her ‘‘psychogenic’’ syncope. Key Point: Apparent loss of consciousness may be psychologic in origin, especially with a history of abuse, stressful www.acsm-csmr.org

incident, or anxiety/depression. Most athletes will improve with psychotherapy. Ninety seconds of ‘‘seizure-like’’ activity is much longer that the hypoxia-induced convulsions of cardiac arrhythmias. Case 3: Football Athlete with Loss of Consciousness after Practice A 19-year-old football player has a witnessed loss of consciousness at the end of practice on an extremely warm August day. He was walking to the locker room when he was noted to wander away from the team briefly before falling to the ground in a prone position. His teammates were the first on the scene, and they described him as being clearly unconscious. He then had a brief stiffening of all four extremities that lasted 15 s. He was unconscious for nearly 5 min in total, during which time, he was noted to have sparse, shallow breathing. Upon regaining consciousness, he was combative for 5 min and acutely disoriented for 25 min. He was taken to a local emergency room via emergency medical services where he was found to be fully oriented, tired, and diffusely sore upon arrival, almost 40 min after the initial loss of consciousness. A head CT in the emergency room yielded normal results, as were basic serum laboratory evaluations. He was discharged home with a normal neurologic examination result, feeling only fatigued and sore. Follow-up MRI and EEG were performed 3 d later, and both yielded normal results. Further history examination revealed no previous similar episodes and no seizure risk factors. He was diagnosed with having a provoked seizure secondary to extreme fatigue, dehydration, and possibly increased core body temperature. He was not started on antiepileptic medication. Key Point: Exertional heat stroke (EHS) should be immediately ruled out on site and immediate on-site cooling should be considered. Preevent confusion, prolonged loss of consciousness (930 s), and prolonged confusion (25 min) are all consistent with seizure and EHS. Both MRI and EEG results are frequently normal in these cases, even if epilepsy is the root cause. In this case, the diagnosis of epilepsy cannot be made, as this is his first seizure and it may have been provoked. Treatment with antiepileptic medications can be considered but is not clearly indicated. References 1. Kapoor W. Syncope. N. Engl. J. Med. 2000; 343:1856Y62. 2. Kosinski D, Grubb BP, Karas BJ, Frederick S. Exercise-induced neurocardiogenic syncope: clinical data, pathophysiological aspects, and potential role of tilt table testing. Europace. 2000; 2:77Y82.

3A. Personal History: Have You Ever Had Excessive Shortness of Breath or Fatigue with Exercise beyond What Is Expected for Your Level of Fitness? Francois Haddad, MD, Gherardo Finocchiaro, MD, and Jonathan Myers, PhD The following questions can help distinguish cardiovascular from pulmonary or other causes of dyspnea. The history along with physical examination and cardiopulmonary studies Current Sports Medicine Reports


257

The Physical Examination A complete neurologic examination should be performed following a suspected neurogenic cause of syncope. Abnormalities of cognition and speech, visual fields, motor strength, sensation, tremor, and gait suggest an underlying neurologic disorder. The Physical Examination If an underlying neurologic disorder is suspected, further study and subspecialty consultation should be considered. If the event was preceded by head trauma with subsequent neurologic deficits, a computed tomography (CT) scan of the head to assess for gross intracranial pathology should be considered, but magnetic resonance imaging (MRI) will yield better resolution for structural or brain tissue pathology. Finally, if seizure activity is suspected, an electroencephalogram (EEG) may be warranted. If the event was preceded by fear, pain, prolonged standing, psychologic stress, or a medical procedure and the athlete has normal physical examination results, the diagnosis of vasovagal syncope or neurally mediated syncope can be made. CASE REPORTS Case 1: Cross-Country Runner with Loss of Consciousness while Running A 15-year-old male cross country runner reported loss of consciousness with exercise during his PPE. Since he was uncertain of the details, his coaches were questioned. They said he stopped running shortly after beginning the second of a two-lap course, had a contusion and laceration to the forehead, and was confused. He denies recall of what happened after he slipped, but the coaches stated that he had fallen, struck his head on a rock, and appeared to have lost consciousness for several seconds. They denied seeing any seizure-like activity. Key Point: Concussions can occur in sports other than traditional ‘‘contact/collision’’ sports and are a common cause of loss of consciousness in athletes. Case 2: Track Athlete with Loss of Consciousness during a Race A 21-year-old female track runner reported loss of consciousness during a 200-m race during her PPE. Her coaches said she was prone and unresponsive on the track, with tremulous activity in both upper and lower extremities lasting about 90 s, after which she was responsive and aware of her situation. She reported having three similar episodes while in high school. There were no other significant personal or family medical history, medication, or supplement use. Neurologic examination result was normal except for the transient inability to move her left arm. Brain imaging (CT and MRI) results were normal. When questioned about stress, she stated she had a previous injury, which occurred in the middle of a championship 200-m race, and she worried constantly about being injured again and unable to compete. Following 48 h of cognitive behavioral therapy, she improved and was able to move all extremities. She was diagnosed with conversion disorder as the cause of her ‘‘psychogenic’’ syncope. Key Point: Apparent loss of consciousness may be psychologic in origin, especially with a history of abuse, stressful www.acsm-csmr.org

incident, or anxiety/depression. Most athletes will improve with psychotherapy. Ninety seconds of ‘‘seizure-like’’ activity is much longer that the hypoxia-induced convulsions of cardiac arrhythmias. Case 3: Football Athlete with Loss of Consciousness after Practice A 19-year-old football player has a witnessed loss of consciousness at the end of practice on an extremely warm August day. He was walking to the locker room when he was noted to wander away from the team briefly before falling to the ground in a prone position. His teammates were the first on the scene, and they described him as being clearly unconscious. He then had a brief stiffening of all four extremities that lasted 15 s. He was unconscious for nearly 5 min in total, during which time, he was noted to have sparse, shallow breathing. Upon regaining consciousness, he was combative for 5 min and acutely disoriented for 25 min. He was taken to a local emergency room via emergency medical services where he was found to be fully oriented, tired, and diffusely sore upon arrival, almost 40 min after the initial loss of consciousness. A head CT in the emergency room yielded normal results, as were basic serum laboratory evaluations. He was discharged home with a normal neurologic examination result, feeling only fatigued and sore. Follow-up MRI and EEG were performed 3 d later, and both yielded normal results. Further history examination revealed no previous similar episodes and no seizure risk factors. He was diagnosed with having a provoked seizure secondary to extreme fatigue, dehydration, and possibly increased core body temperature. He was not started on antiepileptic medication. Key Point: Exertional heat stroke (EHS) should be immediately ruled out on site and immediate on-site cooling should be considered. Preevent confusion, prolonged loss of consciousness (930 s), and prolonged confusion (25 min) are all consistent with seizure and EHS. Both MRI and EEG results are frequently normal in these cases, even if epilepsy is the root cause. In this case, the diagnosis of epilepsy cannot be made, as this is his first seizure and it may have been provoked. Treatment with antiepileptic medications can be considered but is not clearly indicated. References 1. Kapoor W. Syncope. N. Engl. J. Med. 2000; 343:1856Y62. 2. Kosinski D, Grubb BP, Karas BJ, Frederick S. Exercise-induced neurocardiogenic syncope: clinical data, pathophysiological aspects, and potential role of tilt table testing. Europace. 2000; 2:77Y82.

3A. Personal History: Have You Ever Had Excessive Shortness of Breath or Fatigue with Exercise beyond What Is Expected for Your Level of Fitness? Francois Haddad, MD, Gherardo Finocchiaro, MD, and Jonathan Myers, PhD The following questions can help distinguish cardiovascular from pulmonary or other causes of dyspnea. The history along with physical examination and cardiopulmonary studies Current Sports Medicine Reports


257

will generally determine the underlying cause of excessive shortness of breath. Table 1 summarizes different causes of dyspnea important to consider in athletes, and Table 2 summarizes ancillary studies to consider during the workup of dyspnea. 1) Is the shortness of breath recent, or has it been occurring for some time (weeks or months)? Is it consistent and reproducible in its presentation and level of exercise, or is it inconsistent? Shortness of breath with intense exercise is a normal response, but differentiating between normal exerciseinduced shortness of breath and dyspnea associated with a serious heart or lung condition is not always simple (Table 1). A sudden onset, a significant decrease in exercise performance, or an inconsistent presentation may be the first sign of cardiac or pulmonary abnormality and warrants further evaluation. 2) Is there associated chest discomfort? Any athlete with chest pain and dyspnea at a minimum should receive a resting electrocardiogram (ECG), possibly followed by an echocardiogram and a maximal symptomlimited exercise test, provided that the athlete has had no obvious pulmonary or musculoskeletal cause. 3) Is there lightheadedness, or has the patient passed out during exercise? Shortness of breath can be associated with rhythm disturbances during exercise. Any history of lightheadedness or passing out (syncope) should be thoroughly evaluated for the possibility of serious rhythm disturbances. Structural heart diseases associated with these symptoms include congenital aortic stenosis, hypertrophic cardiomyopathy (HCM), or rarely pulmonary hypertension. 4) Is there a family history of a serious heart condition or sudden death? A family history of HCM or arrhythmogenic right ventricular cardiomyopathy strongly raises the likelihood of dyspnea caused by rhythm disturbances, and further investigation based on the details of the family history is indicated (see the family history section). 5) Is there a history of exercise-induced asthma (EIA), or has the individual recently moved to a new environment? EIA is the most common reason for excessive shortness of breath in an otherwise healthy athlete. 6) Is the athlete underperforming during competition and/ or experiencing excessive fatigue during regular activities? Overtraining syndrome (OTS) should be suspected in an athlete who is undergoing heavy training or competition, and cardiovascular causes and other medical illnesses have been ruled out. OTS may be associated with chronic tiredness, unusual fatigue, or shortness of breath with normal athletic competition, underperformance, and difficulty sleeping. Loss of appetite, weight loss, heavy painful muscles, and excessive sweating may be reported. EXERCISE TESTING A cardiopulmonary exercise test can help differentiate between a cardiac and pulmonary cause of excessive shortness of breath with exercise. A cardiac cause should be suspected if the breathing reserve (maximal voluntary ventilation at rest divided by maximal ventilation with exercise) is normal 258


(20% to 40%). Undiagnosed exercise-related asthma can be induced with exercise stress testing that includes the respiratory components. CASE REPORTS Case 1: Dyspnea Associated with a Presyncopal Episode in a Young Athlete A 19-year-old female endurance athlete mentioned on her PPE an episode of dyspnea associated with presyncope while running on a warm day. The athlete had a remote history of chest pain, no palpitations, and no family history of cardiomyopathy or sudden death. Physical examination results were unrevealing. ECG and echocardiogram results were both normal. The athlete was cleared to restart training, and the episode was considered to be neurocardiogenic in origin. Key Point: All athletes with dyspnea and syncope/ presyncope should receive a workup that includes an ECG and resting echocardiogram. Abnormal cardiac remodeling should lead to further evaluation. In athletes, the echocardiogram protocol should include a basal short axis view to visualize the origin of the coronary arteries. Case 2: An Unsuspecting Case of Systemic Illness A 55-year-old national-level cyclist reported feeling shortness of breath during competition. For the last 5 years, he consistently finished in the top three for his age category, but during the last year, he finished in the mid-teens. After initial consultation, his ECG, echocardiogram, and exercise Table 1. Nonpulmonary causes of dyspnea in the athlete.

Cause

Comment

Musculoskeletal injury

Muscle strain, trauma, costochondritis

Myocardial ischemia

Rare cause of dyspnea, but always consider anomalous coronary origin in the young

Other structural cardiac causes

Myocarditis, pericarditis or congenital heart disease (i.e., congenital aortic stenosis limiting exercise performance), or pulmonary hypertension

Arrhythmias

Supraventricular tachycardia can occur in an athlete and cause exertional dyspnea

OTS

Associated with underperforming, waning energy, inadequate rest, and undernutrition for activity level

Chronic fatigue syndrome

Associated with chronic tiredness, excessive fatigue usually following a viral illness

Systemic illness

Anemia, infectious mononucleosis, or systemic illness should be ruled in the presence of unexplained dyspnea or fatigue



Table 2. Suggested investigation for dyspnea in athletes.

Rest Exercise Event Echocardiogram Testing Monitor Others, Comment

Characteristic of Dyspnea

ECG

Mild isolated dyspnea

X (usually)

Consider spirometry

Severe dyspnea

X

X

Consider

Spirometry, CPET may help identify cause of dyspnea

Chest pain without trauma

X

X

X

Symptom limited maximal exercise testing if exertional pain Computerized tomography angiography may be considered if anomalous origin is suspected.

Family history of cardiomyopathy

X

X

Presyncope or syncope

X

X

Excessive fatigue

X

X

echocardiogram results were considered normal, and the patient was advised to resume training. During the following year, his performance continued to drop further. A repeat echocardiogram revealed an increase in wall thickness, and an infiltrative cardiac process was suspected. Further investigation confirmed that the patient had multiple myeloma complicated by cardiac amyloidosis. Key Point: Although a rare case, he highlights the fact that symptoms may be overlooked or underestimated in older athletes. Case 3: Dyspnea and Pulmonary Hypertension in a Young Athlete A young 23-year-old female rower reported progressively increasing shortness of breath during the last year with decreased exercise performance. History was unrevealing, and the physical examination result demonstrated increased second heart sound with a mild holosystolic murmur at the parasternal border. The ECG revealed borderline right axis deviation. Because of the history of progressive dyspnea and the finding on physical examination of an increased S2, an echocardiogram was ordered and results revealed a mildly enlarged right ventricle with mild tricuspid regurgitation, an estimated right ventricular systolic pressure of 42 mm Hg, and normal left ventricle size. Magnetic resonance imaging (MRI) did not show fatty infiltration. Right heart catheterization confirmed the presence of pulmonary arterial hypertension. Key Point: Pulmonary hypertension is a rare cause of dyspnea in athletes but should be considered. An MRI should be obtained in patients with evidence of disproportionate right heart enlargement, especially in nonendurance sports.

3B. Personal History: Have You Ever Had Excessive Shortness of Breath or Fatigue with Exercise beyond What Is Expected for Your Level of Fitness? Jon-Emile S. Kenny, MD and Stephen Ruoss, MD The following questions can help distinguish cardiovascular (CV) from pulmonary or other causes of dyspnea: www.acsm-csmr.org

MRI if ARVD or structural abnormality X

X

Exercise testing may be second line. CBC, TSH, metabolic panel, EBV and CMV titers

1. Are there associated non-pulmonary symptoms such as palpitations, pre-syncope, and/or chest pain? The presence of cardiac-associated symptoms should prompt evaluation for processes including exercise-induced pulmonary arterial hypertension, cardiomyopathies, and valvular abnormalities. 2. Does dyspnea occur suddenly and unexpectedly, or does it occur regularly and predictably with exercise? Intrinsic pulmonary causes of excessive dyspnea with exercise should occur with nearly every episode of exertion. An exception to this statement might be a particular environmental trigger (e.g., cold air) that occurs intermittently with exercise. Truly paroxysmal episodes of excessive dyspnea should raise suspicion for nonpulmonary diseases manifesting as paroxysmal dyspnea, including cardiac arrhythmias or hyperventilation syndrome. 3. When does dyspnea occur during exercise? The onset of dyspnea at peak exercise may suggest a CVrelated arrhythmia or exercise-induced vocal cord dysfunction (VCD) or laryngeal spasm; symptoms often regress as exercise intensity is reduced. In contrast, exercise-induced bronchoconstriction (EIB) is reliably most intense at 10 to 15 min of maximal exercise and tends to resolve gradually over 1 h of sustained exercise. Repeat exercise bouts within 4 h of initial symptoms tend to reduce dyspnea in EIB. 4. Is there audible wheezing or stridor during the dyspnea episode, and does it occur with inspiration, expiration, or both? Audible wheezing during expiration is a strong clinical clue for EIB. However, if noticed during inspiration (stridor), especially if there is no expiratory wheeze and the sounds are associated with hoarseness, VCD should be considered. 5. Are there environmental triggers of dyspnea beyond that of exercise? Allergic individuals are more likely to have EIB and/or exercise-induced asthma. Typical asthmatic triggers such as pollens, cold air, molds, air pollution, animal dander, cigarette or wood smoke, and others can suggest bronchospasm as the underlying etiology. 6. Is there an associated cough, and is it productive? The presence of a nonproductive cough during exercise may be a result of bronchoconstriction. However, cough occurring in the absence of exercise or when not exposed to an environmental trigger may suggest intrinsic airway Current Sports Medicine Reports


259

Table 2. Suggested investigation for dyspnea in athletes.

Rest Exercise Event Echocardiogram Testing Monitor Others, Comment

Characteristic of Dyspnea

ECG

Mild isolated dyspnea

X (usually)

Consider spirometry

Severe dyspnea

X

X

Consider

Spirometry, CPET may help identify cause of dyspnea

Chest pain without trauma

X

X

X

Symptom limited maximal exercise testing if exertional pain Computerized tomography angiography may be considered if anomalous origin is suspected.

Family history of cardiomyopathy

X

X

Presyncope or syncope

X

X

Excessive fatigue

X

X

echocardiogram results were considered normal, and the patient was advised to resume training. During the following year, his performance continued to drop further. A repeat echocardiogram revealed an increase in wall thickness, and an infiltrative cardiac process was suspected. Further investigation confirmed that the patient had multiple myeloma complicated by cardiac amyloidosis. Key Point: Although a rare case, he highlights the fact that symptoms may be overlooked or underestimated in older athletes. Case 3: Dyspnea and Pulmonary Hypertension in a Young Athlete A young 23-year-old female rower reported progressively increasing shortness of breath during the last year with decreased exercise performance. History was unrevealing, and the physical examination result demonstrated increased second heart sound with a mild holosystolic murmur at the parasternal border. The ECG revealed borderline right axis deviation. Because of the history of progressive dyspnea and the finding on physical examination of an increased S2, an echocardiogram was ordered and results revealed a mildly enlarged right ventricle with mild tricuspid regurgitation, an estimated right ventricular systolic pressure of 42 mm Hg, and normal left ventricle size. Magnetic resonance imaging (MRI) did not show fatty infiltration. Right heart catheterization confirmed the presence of pulmonary arterial hypertension. Key Point: Pulmonary hypertension is a rare cause of dyspnea in athletes but should be considered. An MRI should be obtained in patients with evidence of disproportionate right heart enlargement, especially in nonendurance sports.

3B. Personal History: Have You Ever Had Excessive Shortness of Breath or Fatigue with Exercise beyond What Is Expected for Your Level of Fitness? Jon-Emile S. Kenny, MD and Stephen Ruoss, MD The following questions can help distinguish cardiovascular (CV) from pulmonary or other causes of dyspnea: www.acsm-csmr.org

MRI if ARVD or structural abnormality X

X

Exercise testing may be second line. CBC, TSH, metabolic panel, EBV and CMV titers

1. Are there associated non-pulmonary symptoms such as palpitations, pre-syncope, and/or chest pain? The presence of cardiac-associated symptoms should prompt evaluation for processes including exercise-induced pulmonary arterial hypertension, cardiomyopathies, and valvular abnormalities. 2. Does dyspnea occur suddenly and unexpectedly, or does it occur regularly and predictably with exercise? Intrinsic pulmonary causes of excessive dyspnea with exercise should occur with nearly every episode of exertion. An exception to this statement might be a particular environmental trigger (e.g., cold air) that occurs intermittently with exercise. Truly paroxysmal episodes of excessive dyspnea should raise suspicion for nonpulmonary diseases manifesting as paroxysmal dyspnea, including cardiac arrhythmias or hyperventilation syndrome. 3. When does dyspnea occur during exercise? The onset of dyspnea at peak exercise may suggest a CVrelated arrhythmia or exercise-induced vocal cord dysfunction (VCD) or laryngeal spasm; symptoms often regress as exercise intensity is reduced. In contrast, exercise-induced bronchoconstriction (EIB) is reliably most intense at 10 to 15 min of maximal exercise and tends to resolve gradually over 1 h of sustained exercise. Repeat exercise bouts within 4 h of initial symptoms tend to reduce dyspnea in EIB. 4. Is there audible wheezing or stridor during the dyspnea episode, and does it occur with inspiration, expiration, or both? Audible wheezing during expiration is a strong clinical clue for EIB. However, if noticed during inspiration (stridor), especially if there is no expiratory wheeze and the sounds are associated with hoarseness, VCD should be considered. 5. Are there environmental triggers of dyspnea beyond that of exercise? Allergic individuals are more likely to have EIB and/or exercise-induced asthma. Typical asthmatic triggers such as pollens, cold air, molds, air pollution, animal dander, cigarette or wood smoke, and others can suggest bronchospasm as the underlying etiology. 6. Is there an associated cough, and is it productive? The presence of a nonproductive cough during exercise may be a result of bronchoconstriction. However, cough occurring in the absence of exercise or when not exposed to an environmental trigger may suggest intrinsic airway Current Sports Medicine Reports


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disease. If coughing is productive and persistent, diagnoses such as bronchiectasis should be considered. PHYSICAL EXAMINATION Inspect the chest for bony abnormalities such as kyphoscoliosis and pectus excavatum, both of which may lead to exercise limitation, and for symmetry of the posterior chest wall during a normal inspiration as deviation of the chest wall excursion may suggest advanced pleural filling process. Also, inspect the hands for clubbing and cyanosis for evidence of chronic cardiopulmonary conditions. Auscultate for expiratory wheezing that suggests asthma. The episodic nature of asthma makes this examination finding poorly sensitive. Focal findings may indicate localized airway abnormalities such as bronchiectasis or obstruction of the airway lumen by a foreign body. Inspiratory squeaks should be differentiated from upper airway inspiratory stridor by listening over the trachea, as the former typifies bronchiolitis, while the latter, paradoxical VCD. CASE EXAMPLES Case 1: An Amateur Male Cyclist A 33-year-old cyclist presents with excessive dyspnea while exercising over the last 6 months. He notes that his symptoms have progressed since moving to a new location because of his job as an information technology consultant. He has no personal history of allergy. His symptoms are most pronounced shortly into his cycling routine but seem to plateau as he continues to ride, and his return rides are seemingly less symptomatic. Office spirometry is normal; however, exercise spirometry reveals 15% reduction in Forced Expiratory Volume (FEV1) during the first 10 min of cycling and the diagnosis of EIB is made. Key Point: The presence of a refractory period wherein dyspnea is mitigated within 4 h of symptoms onset suggests EIB; most importantly, exercise spirometry that reveals a drop in FEV1 from a normal baseline confirms the diagnosis. Case 2: Teenage Football Player A 16-year-old adolescent male presents with worsening dyspnea during his football tryout routines at high school. The symptoms have been slowly progressive over the preceding years despite utilization of both an inhaled shortacting bronchodilator and inhaled corticosteroid. His symptoms are particularly pronounced at peak exercise and gradually resolve as he stands on the sidelines. His physical examination result is normal, but postexercise office spirometry exhibits and inspiratory flow ‘‘cutoff.’’ Referral to an otolaryngologist for direct laryngoscopy confirms the diagnosis of paradoxical vocal cord movement with inspiration. Key Point: VCD can mimic EIB, and the diagnosis should be confirmed before using bronchodilators for extended periods. A referral to speech pathology is warranted. Case 3: Adult Male Marathon Runner A 43-year-old marathon runner is referred for excessive dyspnea while running. His symptoms have progressed for over 3 years; he notes following an admission for a severe pneumonia. While not exercising, he has complained of 260


productive cough with yellow sputum but no fevers and no chills. He has attributed the cough to his severe postnasal drip that is exacerbated by seasonal allergies. Physical examination result is remarkable for right lower lobe rhonchi with localized inspiratory squeaks. A subsequent chest computerized tomography confirms an area of focal right lower lobe bronchiectasis, and he is referred to a pulmonologist for further evaluation. Key Point: Associated productive cough with localized physical examination findings suggest focal airway disease. Case 4: Young Female Physician Who Works Out A 27-year-old medicine resident is referred for worsening dyspnea during her normal exercise routine along the beach. The dyspnea has worsened as the days have become short in the fall months; her inability to exercise generates a considerable amount of frustration. Her dyspnea begins almost immediately as she runs and does not abate although she notes no wheezing. She has no history of allergy. Her examination, spirometry, and exercise spirometry results are all normal. She is referred for cardiopulmonary exercise testing, and she is noted to have the onset of rapid shallow breathing in anticipation of the test, which continues throughout testing. Her minute ventilation on exercise testing is dramatically elevated. She is referred to a counselor for anxiety management. Key Point: Anxiety can present with asthma-like symptoms. Reference 1. Crapo RO, Casaburi R, Coates AL, et al. Guidelines for methacholine and exercise challenge testing-1999. This official statement of the American Thoracic Society was adopted by the ATS Board of Directors, July 1999. Am. J. Resp. Crit. Care. Med. 2000; 161:309Y29.

Heart Murmurs in Athletes: From Personal History and Physical Examination Sections Shirin Zarafshar, MD and Vic Froelicher, MD 4A. PERSONAL HISTORY: HAVE YOU BEEN TOLD THAT YOU HAVE A HEART MURMUR? To determine the importance of a positive answer to this question, the following questions should be asked: 1. Has the murmur been present since birth, and is it still present? Athletes with congenital murmurs may remain asymptomatic for life, particularly if there is no associated pathologic condition. However, ventricular or atrial septal defects, patent ductus arteriosus (PDA), bicuspid aortic valves, and other causes of heart murmur may have long-term consequences and require correction. 2. Has anyone told you that the murmur is physiologic or pathologic? A documented prior medical evaluation, particularly if imaging has been performed, can facilitate decisions regarding athletic participation. If the studies are of questionable quality or not available, reevaluation may be necessary. Cardiovascular Preparticipation Evaluation


disease. If coughing is productive and persistent, diagnoses such as bronchiectasis should be considered. PHYSICAL EXAMINATION Inspect the chest for bony abnormalities such as kyphoscoliosis and pectus excavatum, both of which may lead to exercise limitation, and for symmetry of the posterior chest wall during a normal inspiration as deviation of the chest wall excursion may suggest advanced pleural filling process. Also, inspect the hands for clubbing and cyanosis for evidence of chronic cardiopulmonary conditions. Auscultate for expiratory wheezing that suggests asthma. The episodic nature of asthma makes this examination finding poorly sensitive. Focal findings may indicate localized airway abnormalities such as bronchiectasis or obstruction of the airway lumen by a foreign body. Inspiratory squeaks should be differentiated from upper airway inspiratory stridor by listening over the trachea, as the former typifies bronchiolitis, while the latter, paradoxical VCD. CASE EXAMPLES Case 1: An Amateur Male Cyclist A 33-year-old cyclist presents with excessive dyspnea while exercising over the last 6 months. He notes that his symptoms have progressed since moving to a new location because of his job as an information technology consultant. He has no personal history of allergy. His symptoms are most pronounced shortly into his cycling routine but seem to plateau as he continues to ride, and his return rides are seemingly less symptomatic. Office spirometry is normal; however, exercise spirometry reveals 15% reduction in Forced Expiratory Volume (FEV1) during the first 10 min of cycling and the diagnosis of EIB is made. Key Point: The presence of a refractory period wherein dyspnea is mitigated within 4 h of symptoms onset suggests EIB; most importantly, exercise spirometry that reveals a drop in FEV1 from a normal baseline confirms the diagnosis. Case 2: Teenage Football Player A 16-year-old adolescent male presents with worsening dyspnea during his football tryout routines at high school. The symptoms have been slowly progressive over the preceding years despite utilization of both an inhaled shortacting bronchodilator and inhaled corticosteroid. His symptoms are particularly pronounced at peak exercise and gradually resolve as he stands on the sidelines. His physical examination result is normal, but postexercise office spirometry exhibits and inspiratory flow ‘‘cutoff.’’ Referral to an otolaryngologist for direct laryngoscopy confirms the diagnosis of paradoxical vocal cord movement with inspiration. Key Point: VCD can mimic EIB, and the diagnosis should be confirmed before using bronchodilators for extended periods. A referral to speech pathology is warranted. Case 3: Adult Male Marathon Runner A 43-year-old marathon runner is referred for excessive dyspnea while running. His symptoms have progressed for over 3 years; he notes following an admission for a severe pneumonia. While not exercising, he has complained of 260


productive cough with yellow sputum but no fevers and no chills. He has attributed the cough to his severe postnasal drip that is exacerbated by seasonal allergies. Physical examination result is remarkable for right lower lobe rhonchi with localized inspiratory squeaks. A subsequent chest computerized tomography confirms an area of focal right lower lobe bronchiectasis, and he is referred to a pulmonologist for further evaluation. Key Point: Associated productive cough with localized physical examination findings suggest focal airway disease. Case 4: Young Female Physician Who Works Out A 27-year-old medicine resident is referred for worsening dyspnea during her normal exercise routine along the beach. The dyspnea has worsened as the days have become short in the fall months; her inability to exercise generates a considerable amount of frustration. Her dyspnea begins almost immediately as she runs and does not abate although she notes no wheezing. She has no history of allergy. Her examination, spirometry, and exercise spirometry results are all normal. She is referred for cardiopulmonary exercise testing, and she is noted to have the onset of rapid shallow breathing in anticipation of the test, which continues throughout testing. Her minute ventilation on exercise testing is dramatically elevated. She is referred to a counselor for anxiety management. Key Point: Anxiety can present with asthma-like symptoms. Reference 1. Crapo RO, Casaburi R, Coates AL, et al. Guidelines for methacholine and exercise challenge testing-1999. This official statement of the American Thoracic Society was adopted by the ATS Board of Directors, July 1999. Am. J. Resp. Crit. Care. Med. 2000; 161:309Y29.

Heart Murmurs in Athletes: From Personal History and Physical Examination Sections Shirin Zarafshar, MD and Vic Froelicher, MD 4A. PERSONAL HISTORY: HAVE YOU BEEN TOLD THAT YOU HAVE A HEART MURMUR? To determine the importance of a positive answer to this question, the following questions should be asked: 1. Has the murmur been present since birth, and is it still present? Athletes with congenital murmurs may remain asymptomatic for life, particularly if there is no associated pathologic condition. However, ventricular or atrial septal defects, patent ductus arteriosus (PDA), bicuspid aortic valves, and other causes of heart murmur may have long-term consequences and require correction. 2. Has anyone told you that the murmur is physiologic or pathologic? A documented prior medical evaluation, particularly if imaging has been performed, can facilitate decisions regarding athletic participation. If the studies are of questionable quality or not available, reevaluation may be necessary. Cardiovascular Preparticipation Evaluation


3. Are there other associated symptoms such as chest pain, shortness of breath, dyspnea on exertion, and/or palpitations? The presence of cardiovascular symptoms such as chest pain, dyspnea, and palpitations can be indicative of an underlying cardiac pathology. Chest pain due to microvascular disease is a feature of HCM. 5. Does anyone in your family have a heart murmur? Familial HCM is an autosomal-dominant trait and one of the most common causes of sudden death in athletes. Other congenital abnormalities that cause murmurs such as Marfan syndrome can cluster in families as well.

PHYSICAL EXAMINATION: IS A HEART MURMUR PRESENT ON PHYSICAL EXAMINATION? The Cardiovascular Examination of the Athlete Setting The cardiac examination should be performed in a stable state and not in the recovery period from an extended period of exercise or competition and in a quiet, comfortable setting with attention given to privacy and room temperature. The examination should be conducted sitting and supine with maneuvers like the Valsalva maneuver, standing, and squatting added if a significant murmur is detected. Normal findings Physiologic and morphologic adaptations of the aerobically trained athlete’s heart include slow heart rate, systolic ejection flow murmur at the upper, left sternal border, a third heart sound, lateral displacement of point of maximal impulse, and hyperdynamic carotid pulses. Athletes predominantly engaged in isometric training (weightlifters) will not exhibit these changes. The examination of the athlete can be challenging because of these physiologic adaptations. Cardiac enlargement by visual examination (apical movements) and palpation (lateral displacement) can simulate the examination of patients with cardiomyopathies or heart failure. The loudest third heart sounds you will ever hear will be in athletes, and they are physiologic when occurring at low heart rates. This contrasts to the third heart sounds in patients with heart failure where they occur at fast heart rates (called ‘‘gallops’’ because of the cadence). Furthermore, third heart sounds in athletes are not related to position, while in patients with heart disease, the S3 gallop is loudest in the supine position. Even in the resting state, systolic flow murmurs can be appreciated in athletes, but they are soft, occur early in systole, and radiate upward rather than laterally toward the apex. While fixed splitting of the heart sounds can be appreciated in the supine position in nonathletes, this splitting is particularly noticeable in athletes. Thus, the finding of fixed splitting should only be considered abnormal if heard in the sitting or standing position. Abnormal findings Any diastolic murmur is abnormal whether or not there is an accompanying systolic murmur. The most common cause is aortic insufficiency (or regurgitation, abbreviated AI or AR). Early AR is best appreciated at the upper left of the sternum, with the athlete in the seated www.acsm-csmr.org

position, during forced expiration and leaning forward. A trick is to carefully listen after the second heart sound to focus attention at the correct time. Since AR can be difficult to appreciate, it is helpful to listen even more carefully when the following occur:

1. A systolic murmur. 2. Wide pulse pressure (950 mm Hg) or a diastolic pressure of 50 mm Hg or less. 3. Hyperdynamic carotid pulse. While AR is the most common cause of a to-and-fro (systolic/diastolic) murmur in athletes, another cause is PDA. This is a congenital abnormality more often encountered than other congenital abnormalities in athletes because athletic performance can be quite normal if the shunt is small. Characteristics that increase the likelihood of systolic murmurs being pathologic include the following:

1. Greater than grade 2/6 intensity 2. Lateral rather than upward radiation 3. Occurrence in mid-systole or late systole rather than in early systole 4. Accompanied by a click in mid-systole or late systole 5. Any murmur that becomes louder with the Valsalva maneuver (best performed supine with your hand on abdomen and telling the athlete to push against your hand while continuing to breathe) Other auscultation abnormalities include a third heart sound during tachycardia or fixed splitting of the second heart sound in the seated position. Sinus tachycardia at rest is abnormal in an aerobically trained athlete, and if not easily explained (recent workout, fever, etc.), further workup may be indicated, particularly if accompanied by any symptoms. CASE EXAMPLES Case 1: Tennis Player with Unexplained Sinus Tachycardia A 20-year-old White female tennis player presented with tachycardia and shortness of breath while going to classes. She normally performed at the top level of competitions but was out of season at the time. She had been symptomatic for nearly a week and presented late in the day after classes. An examination yielded unremarkable results, and she was afebrile, with blood pressure 120/70 and HR of 100 bpm, although S2 seemed to be louder than S1 even at the apex. She was on birth control pills and had just recovered from a leg injury. There was no leg tenderness or swelling. Blood tests were ordered (complete blood count, thyroid function), and a Holter monitor, placed. The Holter was read the next day and was unremarkable except for an average heart rate of 100 bpm with maximum of 140 bpm during light exercise. Laboratory test results were normal. An echocardiogram showed a dilated right ventricle (RV), elevated pulmonary artery (PA) pressures, and normal left ventricle (LV) function. Primary pulmonary hypertension was considered, but she was sent for a computerized tomography chest angiogram lung scan, which revealed pulmonary embolus. She was started on heparin and then treated with Current Sports Medicine Reports


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Coumadin with gradual recovery. She was found to have factor V Leiden genotype. Key Point: Sinus tachycardia may be the only finding with a pulmonary embolus. Case 2: Baseball Player with Continuous Murmur During mass screening, a cardiology fellow noted a systolic murmur while examining a 19-year-old White male baseball player. The athlete was asymptomatic and had normal ECG. The fellow performed screening echocardiogram on site, which was considered normal, and the athlete was cleared for participation. As formality, the supervising cardiologist auscultated the athlete and noted a toand-fro continuous murmur. Echocardiogram and cardiac catheterization revealed PDA with significant right-to-left

shunting. The athlete was offered surgical or percutaneous correction. Key Point: Continuous (systolic and diastolic) and diastolic murmurs are always due to cardiac pathologic lesions. The continuous murmur of PDA is described as a continuous ‘‘washing machine’’ type murmur; best appreciated in the second left intercostal space. A scanning echocardiogram will miss it. Case 3: Soccer Goalie with Fixed Split of Second Heart Sound (S2) A 21-year-old African transfer student complained that he could not ‘‘keep up’’ with his peers during his PPE for collegiate soccer. Upon examination, he had a fixed split S2 while sitting and standing. His ECG results exhibited right axis deviation and incomplete right bundle branch block.

Figure: Major systolic and diastolic murmurs.

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His echocardiogram results appeared to be normal, but a bubble study was added, showing a secundum atrial septal defect permitting an interatrial left-to-right shunt. A rightto-left shunt would have suggested significant pulmonary hypertension and Eisenmenger syndrome. He also had a systolic ejection murmur at the left upper sternal border from increased flow across the pulmonary valve. Catheterization revealed significant shunting with high risk of developing pulmonary hypertension. He was offered surgery or percutaneous closure and opted for the latter. Key Point: Secundum atrial septal defects are the most common type of atrial septal defect (ASD) and the only defects amenable to percutaneous closure. Case 4: Football Lineman with Chest Pain and Systolic Murmur A 20-year-old African-American freshman football lineman was noted to have systolic murmur during PPE. The murmur increased with Valsalva maneuver; his carotid pulse was brisk, and his apical impulse laterally displaced. Upon further questioning, he reported vague exertional chest pain. Echocardiogram results showed mild septal hypertrophy (14 mm), but a magnetic resonance imaging (MRI) demonstrated significant asymmetric hypertrophy at the distal septum. Key Point: The Valsalva maneuver can decrease LV volume and increase mitral flow obstruction to make the diagnosis of HCM with obstruction. Care must be taken to do it properly without breath holding. HCM can be present without such obstruction, and a systolic murmur only be due to hyperdynamic flow not affected by Valsalva. HCM without obstruction can be missed with echocardiography as much as 50% of the time due to focal hypertrophy that is best seen with MRI.

Heart Murmur and Physical Examination in Athletes with a Focus on Congenital Heart Disease George K. Lui, MD; Fahad Al Sindi, MD; and Ami B. Bhatt, MD Congenital heart disease (CHD) is the most common birth defect, occurring in 1 in 100 live births. There is a wide spectrum ranging from small ventricular septal defects (VSD), which spontaneously close before adulthood to complex disease states that require lifelong cardiac care. Children with history of CHD were restricted from exercise until studies demonstrated how exercise can have cardiovascular and quality of life benefits for individuals with surgical repairs (1,2). Because of the advances in diagnosis and surgical repair, the prevalence of adult survivors with CHD continues to grow at a pace of 5% per year, so that there are more than 1 million adults with CHD in the United States (4). These patients often perceive themselves to be ‘‘cured’’ because of surgical repair at a young age. As these individuals become adolescents and adults, they often seek medical advice regarding exercise and sports. Some individuals, however, continue to have reduced exercise www.acsm-csmr.org

capacity from residual abnormalities, and others live with fear and anxiety regarding physical activity. They are often lost to cardiac follow-up and present in adulthood with complications related to their prior repair. It is important to evaluate any patient with history of surgical repair before prescribing exercise. Remember that there are many forms of CHD that present initially in adulthood. KEY POINTS IN THE HISTORICAL ASSESSMENT Simple Defects Simple types of CHD include repaired and unrepaired problems shown in the Table. Patients with repaired defects without residual shunt or pulmonary hypertension can participate in all sports without restriction. Patients with unrepaired defects such as a small-to-moderate atrial septal defect (ASD) or patent ductus arteriosus (PDA) are typically asymptomatic. Late symptoms include dyspnea on exertion or palpitations, and these patients will have history of a murmur. Arrhythmia, heart failure, and pulmonary hypertension are late complications. These patients will need to be referred for further imaging and closure of the shunt before participation in physical activity. Moderate/Complex Defects Moderate-to-complex types of CHD are outlined in the Table. Initial presentation of these lesions in adulthood is rare. Athletes may complain of dyspnea on exertion, dizziness with exertion, palpitations, or syncope. They may have history of a heart murmur, cyanosis, or clubbing. Heart failure, arrhythmias, and sudden cardiac death are shared complications of all these defects. For instance, an athlete may complain of dizziness with exertion and be found to have atrioventricular (AV) block with exercise secondary to AV conduction disease commonly seen in congenitally corrected transposition of the great arteries. In this defect, the patient has atrioventricular and ventriculoarterial discordance whereby the morphologic right ventricle is the systemic ventricle. These patients are prone to complete heart block later in life. Another example of de novo presentation of CHD in adulthood includes hypertension in the adolescence or young adult. Coarctation of the aorta is a narrowing of the aortic isthmus at the proximal descending thoracic aorta, which results in hypertension and late complications of aortic aneurysms, heart failure, and sudden death. These patients will need to be referred for further imaging and surgical/percutaneous repair before participation in sports activities. Individuals with repaired coarctation of the aorta and no residual obstruction or aortic dilation can participate in all sports. More commonly, patients with moderate-to-complex CHD will present having been repaired in early childhood. These individuals will seek recommendation regarding the safety of physical activity. It is important to inquire about symptoms and auscultate for heart murmurs. The history assessment should focus on residual abnormalities that are often present in this patient population such as pulmonary regurgitation in patients with repaired tetralogy of Fallot. Patients with no residual shunting, no significant right ventricular volume overload, and no arrhythmias can participate in all sports. Patients with residual right ventricular Current Sports Medicine Reports


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His echocardiogram results appeared to be normal, but a bubble study was added, showing a secundum atrial septal defect permitting an interatrial left-to-right shunt. A rightto-left shunt would have suggested significant pulmonary hypertension and Eisenmenger syndrome. He also had a systolic ejection murmur at the left upper sternal border from increased flow across the pulmonary valve. Catheterization revealed significant shunting with high risk of developing pulmonary hypertension. He was offered surgery or percutaneous closure and opted for the latter. Key Point: Secundum atrial septal defects are the most common type of atrial septal defect (ASD) and the only defects amenable to percutaneous closure. Case 4: Football Lineman with Chest Pain and Systolic Murmur A 20-year-old African-American freshman football lineman was noted to have systolic murmur during PPE. The murmur increased with Valsalva maneuver; his carotid pulse was brisk, and his apical impulse laterally displaced. Upon further questioning, he reported vague exertional chest pain. Echocardiogram results showed mild septal hypertrophy (14 mm), but a magnetic resonance imaging (MRI) demonstrated significant asymmetric hypertrophy at the distal septum. Key Point: The Valsalva maneuver can decrease LV volume and increase mitral flow obstruction to make the diagnosis of HCM with obstruction. Care must be taken to do it properly without breath holding. HCM can be present without such obstruction, and a systolic murmur only be due to hyperdynamic flow not affected by Valsalva. HCM without obstruction can be missed with echocardiography as much as 50% of the time due to focal hypertrophy that is best seen with MRI.

Heart Murmur and Physical Examination in Athletes with a Focus on Congenital Heart Disease George K. Lui, MD; Fahad Al Sindi, MD; and Ami B. Bhatt, MD Congenital heart disease (CHD) is the most common birth defect, occurring in 1 in 100 live births. There is a wide spectrum ranging from small ventricular septal defects (VSD), which spontaneously close before adulthood to complex disease states that require lifelong cardiac care. Children with history of CHD were restricted from exercise until studies demonstrated how exercise can have cardiovascular and quality of life benefits for individuals with surgical repairs (1,2). Because of the advances in diagnosis and surgical repair, the prevalence of adult survivors with CHD continues to grow at a pace of 5% per year, so that there are more than 1 million adults with CHD in the United States (4). These patients often perceive themselves to be ‘‘cured’’ because of surgical repair at a young age. As these individuals become adolescents and adults, they often seek medical advice regarding exercise and sports. Some individuals, however, continue to have reduced exercise www.acsm-csmr.org

capacity from residual abnormalities, and others live with fear and anxiety regarding physical activity. They are often lost to cardiac follow-up and present in adulthood with complications related to their prior repair. It is important to evaluate any patient with history of surgical repair before prescribing exercise. Remember that there are many forms of CHD that present initially in adulthood. KEY POINTS IN THE HISTORICAL ASSESSMENT Simple Defects Simple types of CHD include repaired and unrepaired problems shown in the Table. Patients with repaired defects without residual shunt or pulmonary hypertension can participate in all sports without restriction. Patients with unrepaired defects such as a small-to-moderate atrial septal defect (ASD) or patent ductus arteriosus (PDA) are typically asymptomatic. Late symptoms include dyspnea on exertion or palpitations, and these patients will have history of a murmur. Arrhythmia, heart failure, and pulmonary hypertension are late complications. These patients will need to be referred for further imaging and closure of the shunt before participation in physical activity. Moderate/Complex Defects Moderate-to-complex types of CHD are outlined in the Table. Initial presentation of these lesions in adulthood is rare. Athletes may complain of dyspnea on exertion, dizziness with exertion, palpitations, or syncope. They may have history of a heart murmur, cyanosis, or clubbing. Heart failure, arrhythmias, and sudden cardiac death are shared complications of all these defects. For instance, an athlete may complain of dizziness with exertion and be found to have atrioventricular (AV) block with exercise secondary to AV conduction disease commonly seen in congenitally corrected transposition of the great arteries. In this defect, the patient has atrioventricular and ventriculoarterial discordance whereby the morphologic right ventricle is the systemic ventricle. These patients are prone to complete heart block later in life. Another example of de novo presentation of CHD in adulthood includes hypertension in the adolescence or young adult. Coarctation of the aorta is a narrowing of the aortic isthmus at the proximal descending thoracic aorta, which results in hypertension and late complications of aortic aneurysms, heart failure, and sudden death. These patients will need to be referred for further imaging and surgical/percutaneous repair before participation in sports activities. Individuals with repaired coarctation of the aorta and no residual obstruction or aortic dilation can participate in all sports. More commonly, patients with moderate-to-complex CHD will present having been repaired in early childhood. These individuals will seek recommendation regarding the safety of physical activity. It is important to inquire about symptoms and auscultate for heart murmurs. The history assessment should focus on residual abnormalities that are often present in this patient population such as pulmonary regurgitation in patients with repaired tetralogy of Fallot. Patients with no residual shunting, no significant right ventricular volume overload, and no arrhythmias can participate in all sports. Patients with residual right ventricular Current Sports Medicine Reports


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hypertension or arrhythmias should only participate in lowintensity sports. KEY POINTS IN THE PHYSICAL EXAMINATION 1. Peripheral Examination Blood pressure can be quite helpful in CHD. A large pulse pressure suggests diastolic runoff from PDA, ruptured sinus of Valsalva, or aortic regurgitation, whereas a narrow pulse pressure suggests aortic or left ventricular outflow stenosis. A difference in blood pressure between the arms and legs may suggest coarctation of the aorta or peripheral vascular disease. The presence of cyanosis is always pathologic in CHD and typically represents an intracardiac shunt. Clubbing of the fingers can be seen in cyanotic CHD. Dysmorphic features may alert one to a syndrome such as Turner, Noonan, or Down syndrome. 2. Precordial Examination Upon examination of the chest, scapular scars suggest previous shunt operations and thoracotomy scars are indicative of previous coarctation or PDA surgery. Palpation of the pericardium identifies the location of the apex beat, which can be located on the right in dextrocardia. A palpable thrill may indicate a prominent murmur. The first heart sound (S1) can be loud in mitral stenosis and diminished and ‘‘muffled’’ in mitral regurgitation. The second heart sound (S2) has two components that are physiologically split during inspiration. Fixed splitting is typically seen in an ASD. A loud P2 can be appreciated in pulmonary hypertension. A VSD produces a holosystolic murmur located at the left sternal edge. It is differentiated from mitral regurgitation in that it produces a low-pitch ‘‘harsh’’ sound indicating the high pressure gradient across the defect. Diastolic murmurs such as pulmonary regurgitation can be seen years after tetralogy of Fallot repair. A continuous murmur is typically associated with PDA.

Key Point: Residual hemodynamic abnormalities can occur in patients with history of CHD repair. Case 2: Marathon Runner with Dyspnea on Exertion A 20-year-old marathon runner presents to her family doctor with increased dyspnea on exertion, climbing two flights of stairs. Her heart rate is 98 bpm, with blood pressure of 100/60. Prior auscultation examination revealed a pulmonic click, which decreased with inspiration, but no click was heard now. She has a systolic ejection murmur best heard at the left second intercostal space and no diastolic murmur. Echocardiography confirmed the diagnosis of pulmonary stenosis. Upon physical examination, she has two components of her second heart sound. The P2 is normally split. Her pulmonic click is the only right-sided heart sound, which diminishes with inspiration. When inspiring, the venous return is increased to the heart, causing the highly mobile domed valve, which has been primed to open with less intensity, and thereby creates a softer click. The absence of a diastolic murmur suggests no concomitant pulmonary regurgitation. Percutaneous valvuloplasty relieved the pulmonary stenosis and allowed her to return to marathon running. Key Point: Change in physical examination and presence of heart murmur should prompt further evaluation for CHD. Table. Simple defects ASD Small VSD Small PDA Mild pulmonary stenosis Repaired ASD, VSD, or PDA Moderate/complex defects Atrioventricular canal

Case 1: Soccer Player with Shortness of Breath A 23-year-old woman with history of repaired VSD and subaortic membrane stenosis at age 5 presents with shortness of breath. She has been playing soccer normally for many years without any difficulty until the last year when she had noted that she was more out of breath with exertion. Upon examination, heart rate is 60 bpm, blood pressure is 100/70, and O2sat is 100%. She has a loud systolic ejection murmur at the left upper sternal border with an RV lift and thrill. Electrocardiogram result was within normal limits. An echocardiogram demonstrated prominent muscle bundles at the right ventricular outflow tract with a peak gradient of 125 mm Hg. The right ventricle was hypertrophied and dilated. There was tricuspid regurgitation and no residual VSD or pulmonary hypertension. A cardiac catheterization confirmed double-chamber right ventricle with a proximal RV pressure of 83/15 and low distal RV pressures. The anomalous muscle bundles were resected from the right ventricular outflow tract without complication. She was able to resume sports activity with no further dyspnea. 264


Coarctation of the aorta Double outlet right ventricle Ebstein anomaly Eisenmenger syndrome Hypoplastic left heart syndrome Moderate-to-severe pulmonary stenosis Subaortic stenosis Single ventricle Tetralogy of Fallot Transposition of the great arteries Truncus arteriosus [Adapted from Warnes CA, Williams RG, Bashore TM, et al. ACC/ AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008; 118:e714Ye833. Copyright * 2008. Used with permission.] Cardiovascular Preparticipation Evaluation


References

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1. Dua JS, Cooper AR, Fox KR, Graham Stuart A. Exercise training in adults with congenital heart disease: feasibility and benefits. Int. J. Cardiol. 2010; 138:196Y205.

Guidelines for proper blood pressure measurement.

2. Galioto FM, Tomassoni TL. Exercise rehabilitation in congenital cardiac disease. Progress Pediatr. Cardiol. 1993; 2:50Y4.

Circumstances V Avoid caffeine at least 1 h prior to testing. Calibrate cuff appropriately.

3. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008; 118:e714Y833. 4. Williams RG, Pearson GD, Barst RJ, et al. Report of the National Heart, Lung, and Blood Institute working group on research in adult congenital heart disease. J. Am. Coll. Cardiol. 2006; 47:701Y7.

Hypertension in Athletes from Physical Examination and Personal History Section Aswathnarayan R. Manandhi, MD and Paul D. Thompson, MD, FACC 5. Physical Examination/Personal History: Do You Have or Have You Ever Been Told You Have Hypertention? The goal in measuring blood pressure during the PPE is to reduce the risk of an acute cardiac event during exercise such as an aortic disruption or a cerebral vascular event and to identify athletes in need of further evaluation and possible treatment. Blood pressure in children increases with age and height. Percentile values are available at http://www.nhlbi.nih.gov/ guidelines/hypertension/child_tbl.htm. Children with values 995th percentile are considered to have hypertension, and those between the 90th and 95th percentile are considered to have prehypertension. For children older than 14 years, the 90th percentile is generally exceeded with a systolic blood pressure (SBP) 9130 mm Hg or a diastolic blood pressure (DBP) 980 mm Hg. Hypertension in adults requires SBP and DBP values greater than 140 and 90 mm Hg, respectively. Individuals meeting these criteria on screening should undergo further evaluation. Blood pressure measurements should be made using measurement guidelines (5) (Table) Athletes with normal blood pressure should have their blood pressure measured at every health care encounter. Adults with values of SBP between 120 and 139 mm Hg and of DBP between 80 and 89 mm Hg should be reevaluated in a year. Secondary hypertension is ultimately found in approximately 5% of athletes and physically active individuals (7). The possibility of secondary hypertension should be considered when the history or physical examination raises this possibility and when hypertension is severe, occurs in younger athletes, has a sudden onset, and is not controlled by the usual drug regimen. The history examination should determine whether the athlete uses any over-the-counter (OTC) medications or supplements that could increase blood pressure. These include OTC cold and sinus remedies that contain sympathomimetic agents, nonsteroidal medications frequently used by athlete for pain management, excessive alcohol consumption, www.acsm-csmr.org

Posture V Sit quietly for 5 min prior to BP reading.

Blood pressure cuff size V The bladder should encircle Q80% of the arm circumference, large cuff for arms >33 cm and small cuff for 20 mm Hg above the disappearance of the radial pulse. & If elevated, blood pressure should be measured in both arms and the higher value must be used for diagnosis. & Additional measurements >1 wk apart should be obtained if the initial pressures are elevated.

or the use of cocaine, amphetamines, anabolic steroids, or selective androgen receptor modulators (8). The physical examination should seek to identify secondary causes of hypertension. This should include inspection for the buffalo hump and moon facies of Cushing syndrome and signs of hyperthyroidism, auscultation for abdominal and flank vascular bruits, and simultaneous palpation of the radial and femoral arterial pulse to exclude a radial femoral pulse delay. In adolescents and adults, a radial femoral pulse delay is a better sign of coarctation than absence of the femoral pulse (9). A wide pulse pressure and primarily systolic hypertension should prompt a search for aortic insufficiency. Laboratory testing also is designed to detect secondary causes of hypertension and should include a thyroidstimulating hormone level to detect hyperthyroidism, electrolytes to look for the hypokalemia associated with aldosterone excess, a serum calcium level to detect hyperparathyroidism, measures of renal function, and a urinalysis. A low pretreatment potassium level or rapid decrease in potassium with diuretic therapy suggests hyperaldosteronism. Adrenal hyperplasia without adenoma is a variant of hyperaldosteronism that often occurs in patients with difficult-to-treat hypertension and responds well to aldosterone antagonists.

TREATMENT OF ATHLETES WITH HYPERTENSION Treatment should be aimed at the underlying cause. Physical activity, low-sodium diets, and alcohol restriction can reduce blood pressure in some athletes. Both sodium restriction and alcohol restriction should be tried before initiating drug therapy. Most individuals with carefully diagnosed hypertension will require medication therapy, and approximately two-thirds of patients require more than one agent. We often initiate therapy with a low-dose diuretic followed by either an ACE-I or ARB alone or in combination in non-Blacks (10). Initial treatment in Blacks is either a Current Sports Medicine Reports


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References

Table 1.

1. Dua JS, Cooper AR, Fox KR, Graham Stuart A. Exercise training in adults with congenital heart disease: feasibility and benefits. Int. J. Cardiol. 2010; 138:196Y205.

Guidelines for proper blood pressure measurement.

2. Galioto FM, Tomassoni TL. Exercise rehabilitation in congenital cardiac disease. Progress Pediatr. Cardiol. 1993; 2:50Y4.

Circumstances V Avoid caffeine at least 1 h prior to testing. Calibrate cuff appropriately.

3. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008; 118:e714Y833. 4. Williams RG, Pearson GD, Barst RJ, et al. Report of the National Heart, Lung, and Blood Institute working group on research in adult congenital heart disease. J. Am. Coll. Cardiol. 2006; 47:701Y7.

Hypertension in Athletes from Physical Examination and Personal History Section Aswathnarayan R. Manandhi, MD and Paul D. Thompson, MD, FACC 5. Physical Examination/Personal History: Do You Have or Have You Ever Been Told You Have Hypertention? The goal in measuring blood pressure during the PPE is to reduce the risk of an acute cardiac event during exercise such as an aortic disruption or a cerebral vascular event and to identify athletes in need of further evaluation and possible treatment. Blood pressure in children increases with age and height. Percentile values are available at http://www.nhlbi.nih.gov/ guidelines/hypertension/child_tbl.htm. Children with values 995th percentile are considered to have hypertension, and those between the 90th and 95th percentile are considered to have prehypertension. For children older than 14 years, the 90th percentile is generally exceeded with a systolic blood pressure (SBP) 9130 mm Hg or a diastolic blood pressure (DBP) 980 mm Hg. Hypertension in adults requires SBP and DBP values greater than 140 and 90 mm Hg, respectively. Individuals meeting these criteria on screening should undergo further evaluation. Blood pressure measurements should be made using measurement guidelines (5) (Table) Athletes with normal blood pressure should have their blood pressure measured at every health care encounter. Adults with values of SBP between 120 and 139 mm Hg and of DBP between 80 and 89 mm Hg should be reevaluated in a year. Secondary hypertension is ultimately found in approximately 5% of athletes and physically active individuals (7). The possibility of secondary hypertension should be considered when the history or physical examination raises this possibility and when hypertension is severe, occurs in younger athletes, has a sudden onset, and is not controlled by the usual drug regimen. The history examination should determine whether the athlete uses any over-the-counter (OTC) medications or supplements that could increase blood pressure. These include OTC cold and sinus remedies that contain sympathomimetic agents, nonsteroidal medications frequently used by athlete for pain management, excessive alcohol consumption, www.acsm-csmr.org

Posture V Sit quietly for 5 min prior to BP reading.

Blood pressure cuff size V The bladder should encircle Q80% of the arm circumference, large cuff for arms >33 cm and small cuff for 20 mm Hg above the disappearance of the radial pulse. & If elevated, blood pressure should be measured in both arms and the higher value must be used for diagnosis. & Additional measurements >1 wk apart should be obtained if the initial pressures are elevated.

or the use of cocaine, amphetamines, anabolic steroids, or selective androgen receptor modulators (8). The physical examination should seek to identify secondary causes of hypertension. This should include inspection for the buffalo hump and moon facies of Cushing syndrome and signs of hyperthyroidism, auscultation for abdominal and flank vascular bruits, and simultaneous palpation of the radial and femoral arterial pulse to exclude a radial femoral pulse delay. In adolescents and adults, a radial femoral pulse delay is a better sign of coarctation than absence of the femoral pulse (9). A wide pulse pressure and primarily systolic hypertension should prompt a search for aortic insufficiency. Laboratory testing also is designed to detect secondary causes of hypertension and should include a thyroidstimulating hormone level to detect hyperthyroidism, electrolytes to look for the hypokalemia associated with aldosterone excess, a serum calcium level to detect hyperparathyroidism, measures of renal function, and a urinalysis. A low pretreatment potassium level or rapid decrease in potassium with diuretic therapy suggests hyperaldosteronism. Adrenal hyperplasia without adenoma is a variant of hyperaldosteronism that often occurs in patients with difficult-to-treat hypertension and responds well to aldosterone antagonists.

TREATMENT OF ATHLETES WITH HYPERTENSION Treatment should be aimed at the underlying cause. Physical activity, low-sodium diets, and alcohol restriction can reduce blood pressure in some athletes. Both sodium restriction and alcohol restriction should be tried before initiating drug therapy. Most individuals with carefully diagnosed hypertension will require medication therapy, and approximately two-thirds of patients require more than one agent. We often initiate therapy with a low-dose diuretic followed by either an ACE-I or ARB alone or in combination in non-Blacks (10). Initial treatment in Blacks is either a Current Sports Medicine Reports


265

thiazide-type diuretic or a calcium channel-blocker alone or in combination (10,11). SPECIAL CONSIDERATIONS IN ATHLETES Diuretics increase the urinary loss of potassium, and hypokalemia can cause muscle cramps and rhabdomyolysis, especially in athletes exercising in hot environments. Nevertheless, we frequently use low-dose diuretics as initial therapy, especially in Blacks since Black athletes are more sensitive to sodium than their White counterparts. We avoid higher doses except in athletes with large body mass and hold the diuretics several days before athletic events that are associated with high sweating rates and loss of body fluid. We often combine diuretics with ACE-I or ARB that prevent hypokalemia produced by diuretics alone. Athletes have used diuretics to dilute the urine, thereby decreasing the concentration of banned ergogenic medications. Diuretics also can be used to ‘‘make weight’’ in sports with weight classes such as wrestling and boxing. Consequently, diuretic use is prohibited by the World Anti-Doping Agency (12). Our second choice for blood pressure control in athletes is either an ACE-I or an ARB because there are theoretical reasons to believe that ACE inhibitors can improve athletic performance and they reduce potassium loss from the diuretic. The most challenging side effect of ACE inhibitor is a dry cough in approximately 10% of patients. ARB can be used in patients who develop a cough (4). A variety of other agents is available including calcium channel blockers. Avoid beta-blocking agents because they impair aerobic performance and their use is banned for athletes participating in precision events (12). These prohibitions exist because beta-blockade slows the heart rate and allows more time between pulse movement to fire a steadier shot in shooting and archery. SUBSEQUENT EVALUATION Athletes treated for hypertension should be seen frequently until their blood pressure is controlled. Patients on diuretics or agents that affect the renin-angiotensin system should have periodic measurements of their serum potassium and creatinine levels. Follow-up visits can be at 3- to 6month intervals once the BP is at target and stable (4). Case 1 Seven of 28 high school football players undergoing a team screening provided free by a local physician were referred for hypertension evaluation. None of the athletes had hypertension when their blood pressure was measured using a mercury sphygmomanometer. Subsequent discussion with the physician revealed that he had used an inappropriately small cuff. All seven athletes were linemen. Key Point: An undersized cuff on a muscular arm produces spuriously elevated blood pressures. Case 2 A 17-year-old high school senior who had recently relocated to Connecticut from northern Maine was referred for hypertension detected during an Air Force physical screening. Upon examination, his blood pressure was 184/ 105 mm Hg in each arm. His cardiovascular examination result was normal, and he had palpable femoral pulses 266


bilaterally, but there was a clear discrepancy between the timing of his radial and femoral pulses. His chest x-ray showed extensive rib notching consistent with aortic coarctation and collateral vessel development. He was referred for surgical repair. Key Point: Femoral pulses are often present in adolescents with previously undetected aortic coarctation because the femoral pulse is well supplied by collaterals. Adolescents and young adults with hypertension should be examined for a radial-femoral pulse delay. Case 3 An 18-year-old high school football defensive lineman was referred for a screening blood pressure of 188/68 mm Hg in each arm. His cardiac examination revealed a 3/6 diastolic murmur over his sternum. His echocardiogram results showed moderate aortic insufficiency. His hypertension was treated with an ARB and was cured. Key Point: Aortic insufficiency produces systolic hypertension and wide pulse pressure. It is often hard to hear, so aortic insufficiency should be excluded in all athletes with systolic hypertension and wide pulse pressure. References 1. Chobanian AV, Bakris GL, Black HR, et al. The seventh report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure: the JNC 7 report. JAMA. 2003; 289:2560Y72. 2. James PA, Oparil S, Carter BL, et al. 2014 evidence-based guideline for the management of high blood pressure in adults: report from the panel members appointed to the Eighth Joint National Committee (JNC 8). JAMA. 2014; 311:507Y20. 3. Pickering TG. Measurement of blood pressure in and out of the office. J. Clin. Hypertens. (Greenwich). 2005;7: 123Y9. 4. World Anti-Doping Agency. The 2013 Prohibited List International Standard. 2013.

EDITORS Irfan M. Asif, MD Vice Chair Research & Academics Director, Sports Medicine Fellowship Associate Professor Department of Family Medicine Greenville Health System/University of South Carolina Greenville [email protected] 864-455-7800 William Roberts, MD, MS Professor, Family Medicine 1414 Maryland Avenue E St. Paul, MN 55106 [email protected] 651-772-3461 Michael Frederickson, MD Professor, Stanford University [email protected] V. F. Froelicher, MD (111C) Professor of Medicine, Stanford University Palo Alto VA Medical Center, 650-493-5000 x 64605 3801 Miranda Avenue, Building 100, Room E2-441 Palo Alto, CA 94304-1207 [email protected] Cardiovascular Preparticipation Evaluation


INVITED PARTICIPANTS Francis G. O’Connor, COL, MC, USA Professor and Chair, Military and Emergency Medicine Medical Director, Consortium for Health and Military Performance Uniformed Services University of the Health Sciences Aaron L. Baggish, MD, FACC Cardiovascular Performance Program Massachusetts General Hospital Meagan M. Wasfy, MD Sports Medicine Fellow Cardiovascular Performance Program Massachusetts General Hospital Ricardo Stein, MD Jeffrey S. Kutcher, MD Associate Professor Director, Michigan NeuroSport Department of Neurology University of Michigan Aswathnarayan R. Manandhi, MD Cardiology Division, Hartford Hospital Paul D. Thompson, MD, FACC Chief of Cardiology, Hartford Hospital Abhimanyu (Manu) Uberoi, MD Cardiology Fellow, Cedars Sinai Medical Center Benjamin D. Levine, MD Director, Institute for Exercise and Environmental Medicine S. Finley Ewing Jr. Chair for Wellness at Texas Health Presbyterian Dallas Harry S. Moss Heart Chair for Cardiovascular Research Professor of Medicine and Cardiology Distinguished Professorship in Exercise Science University of Texas Southwestern Medical Center at Dallas

www.acsm-csmr.org

Chad Asplund, MD, MPH, FACSM Medical Director, Student Health Services and Sports Medicine Associate Professor, Family Medicine Georgia Regents University Team Physician Georgia Regents University, Paine College, Augusta Greenjackets STANFORD FACULTY Jonathan Myers, PhD Palo Alto VA Medical Center David Liang, MD Director, Stanford Marfan’s Clinic Marco Perez, MD Staff Electrophysiologist Stanford CV Genomics Rick Dewey, MD Cardiology Fellow and Genomics Program Researcher Stanford Cardiology Michael J. Khadavi, MD PM&R Resident, Sportsmedicine Program Stanford George K. Lui, MD, FACC Medical Director, The Adult Congenital Heart Program at Stanford Stanford Hospital & Clinics and Lucile Packard Children’s Hospital Clinical Assistant Professor of Medicine Division of Cardiovascular Medicine Stanford University School of Medicine Jon-Emile Stuart Kenny, MD Pulmonary Fellow Stephen Ruoss, MD Professor of Medicine, Pulmonary and Critical Care Stanford University Medical Center



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Erratum The Cardiovascular Preparticipation Evaluation (PPE) for the Primary Care and Sports Medicine Physician, Part I: Erratum In the article appearing on pages 246Y267 of the May/June 2015 issue, the following errors were detected:

& On page 246, column 2, paragraph 2, lines 16Y18, the sentence should read: ‘‘This recent document added two elements regarding palpitations and previous evaluations similar to those in the fourth PPE. The two elements are: prior restriction and prior evaluations (palpitations were added to the dyspnea/fatigue element).’’ & The 12-Step Questionnaire Recommendations for Health Care Providers for Preparticipation Cardiovascular Screening in Athletes, on page 247 contained an error in the Preparticipation Physical Evaluation (edition 4) questions section. The question: ‘‘1. Has a doctor ever denied or restricted your participation in sports for any reason?’’ should have appeared under the heading ‘‘Heart health questions about you.’’ Additionally on page 248 in this same table, the word arachmodactylyl was misspelled. & On page 251, column 1, paragraph 1, line 1, myocardial was misspelled. & On page 256, column 2, point 5, this should read psychological stress. & On page 266, there was a misspelling in the name of Editor Michael Fredericson, MD, FACSM and contact information for editor Victor F. Froelicher, MD, FACSM should have appeared as: Victor F. Froelicher, MD, FACSM Professor of Cardiovascular Medicine and Orthopedics/Sports Medicine Director Sports Cardiology Clinic, Stanford University Center for Inherited Cardiovascular Disease 870 Quarry Road Falk Cardiovascular Research Building; MC-5406/Room CV-285 Stanford, CA 94305-5406 [email protected] Reference 1. Asif IM, Roberts WO, Fredericson M, Froelicher V. The cardiovascular preparticipation evaluation (PPE) for primary care and sports medicine physician, part 1. Curr Sports Med Rep. 2015; 14:246Y67.

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SPECIAL COMMUNICATIONS The Cardiovascular Preparticipation Evaluation (PPE) for the Primary Care and Sports Medicine Physician, Part II Editors: Irfan M. Asif, MD; William O. Roberts, MD, MS, FACSM; Michael Fredericson, MD, FACSM; and Victor F. Froelicher, MD, FACSM This article aims to provide a rational approach to positive responses to the American Heart Association (AHA) 12-Step Questionnaire and fourth edition Preparticipation Physical Evaluation (PPE) monograph for assessing cardiovascular (CV) risk in athletes. This will assist primary care and sports medicine physicians in determining the need for the following:

1. Follow-up questions to a positive response that will enhance the history and help determine whether a condition that puts an athlete at increased CV risk exists. 2. Any basic diagnostic tests to further assess the athlete and that will assist in making an informed decision. 3. The need for a consultation or referral to an appropriate specialist. Our goal is to help the primary care and sports medicine physician with the critical decision making regarding positive responses to the AHA 12-Step Questionnaire and criteria for athlete clearance, as follows:

1. Could this be a potentially lethal problem? 2. Does this need additional workup or just an electrocardiogram (ECG)? 3. Does this require consultation with a specialist (and which specialty)? While there are some differences in the questions from the AHA 12 points and the CV questions in the PPE fourthedition monograph, the underlying intent is the same and the information provided is easily utilized for both question sets. This is the second of this two-part Special Communication. Part 1 published in the May/June 2015 issue of Current Sports Medicine Reports and is available on the journal’s Web site [www.acsm-csmr.org].

Palpitations in Athletes Ramy S. Abdelfattah, MD and Victor F. Froelicher, MD, FACSM Personal History Have you ever experienced palpitations with exercise? The symptom of palpitations in an athlete or perspective athlete can range from benign with no relationship to arrhythmias to malignant with a risk of a fatal arrhythmic event. This awareness of heartbeats could be due to a normal physiological process, an underlying CV pathology, a systemic cause, or a psychological issue. The heartbeat that is felt may be regular or erratic, fast or slow, strong or weak or may feel like a missed or extra beat that can occur during exercise, www.acsm-csmr.org

emotional situations, and stress and sometimes without any provoking factors (5,16). The figure shows common cardiac and noncardiac causes of palpitations (13,20). The variability of clinical outcomes of palpitations, ranging from a single benign event to the first sign of a potentially life-threatening problem, makes the evaluation of palpitations a challenging process (17). In two clinical studies, a clinically significant arrhythmia was diagnosed in one-fifth of patients presenting with palpitations (8,24) while a lower prevalence of pathology is found in healthy young athletes (13). The prevalence of palpitations in athletes varies widely depending on age and type of sports. The fourth edition PPE monograph inquires exclusively about palpitations during exercise, and query regarding exertional palpitations is now included in the 2014 AHA elements for preparticipation evaluation (15). When the answer is positive for exertional palpitations, a careful history examination should be conducted to determine whether further evaluation is needed and which diagnostic testing should be ordered. The critical questions are listed in Table 1. 1) Analysis of the complaint This should start by an open-ended question without prompting but noted in the record. Asking the athlete to tap the rhythm of their palpitations or to choose from rhythms tapped by the physician can be helpful (Table 2) (25). Palpitations associated with syncope or presyncope may signify a clinical significant arrhythmia such as supraventricular or ventricular tachycardia (VT) in athletes (14,29). Palpitations while sitting quietly or lying in bed that last for brief periods and felt as a single skipped beat or a sensation of stopping of the heartbeats and then restarting suggest premature atrial or ventricular extrasystoles (1,29). Palpitations are not likely to be due to a significant cardiac arrhythmia when the onset and termination are gradual (20). For example, a sudden onset and abrupt termination of an irregularly irregular heartbeat reinforced by atrial fibrillation (AF) type tapping out along with weakness and fatigue suggest AF. However, sudden onset of fast regular palpitations that continue after stopping exercise broken by positional change or vagal maneuver that can be associated with polyuria, pounding sensation in the neck, shirt flapping, and/or visible cannon A waves in the neck ‘‘frog sign’’ suggests a supraventricular reentry tachyarrhythmia particularly atrioventricular reentrant tachycardia (AVRT) and atrioventricular nodal reentrant tachycardia (AVNRT) (2,7,11,20). Patients with regular palpitations were found to be twice more likely to have a ‘‘significant’’ cardiac arrhythmia as a cause for their palpitations (24). When palpitations are felt as strong or powerful contractions, but regular and persistent, the physician should think of a systemic cause, such as a high stroke volume or a structural heart disease, such as aortic regurgitation (20). Frequent extra beats, some with super hard beats, some running in a row or associated with weakness or collapse, could be premature ventricular contractions (PVC) or VT. Current Sports Medicine Reports


333

SPECIAL COMMUNICATIONS The Cardiovascular Preparticipation Evaluation (PPE) for the Primary Care and Sports Medicine Physician, Part II Editors: Irfan M. Asif, MD; William O. Roberts, MD, MS, FACSM; Michael Fredericson, MD, FACSM; and Victor F. Froelicher, MD, FACSM This article aims to provide a rational approach to positive responses to the American Heart Association (AHA) 12-Step Questionnaire and fourth edition Preparticipation Physical Evaluation (PPE) monograph for assessing cardiovascular (CV) risk in athletes. This will assist primary care and sports medicine physicians in determining the need for the following:

1. Follow-up questions to a positive response that will enhance the history and help determine whether a condition that puts an athlete at increased CV risk exists. 2. Any basic diagnostic tests to further assess the athlete and that will assist in making an informed decision. 3. The need for a consultation or referral to an appropriate specialist. Our goal is to help the primary care and sports medicine physician with the critical decision making regarding positive responses to the AHA 12-Step Questionnaire and criteria for athlete clearance, as follows:

1. Could this be a potentially lethal problem? 2. Does this need additional workup or just an electrocardiogram (ECG)? 3. Does this require consultation with a specialist (and which specialty)? While there are some differences in the questions from the AHA 12 points and the CV questions in the PPE fourthedition monograph, the underlying intent is the same and the information provided is easily utilized for both question sets. This is the second of this two-part Special Communication. Part 1 published in the May/June 2015 issue of Current Sports Medicine Reports and is available on the journal’s Web site [www.acsm-csmr.org].

Palpitations in Athletes Ramy S. Abdelfattah, MD and Victor F. Froelicher, MD, FACSM Personal History Have you ever experienced palpitations with exercise? The symptom of palpitations in an athlete or perspective athlete can range from benign with no relationship to arrhythmias to malignant with a risk of a fatal arrhythmic event. This awareness of heartbeats could be due to a normal physiological process, an underlying CV pathology, a systemic cause, or a psychological issue. The heartbeat that is felt may be regular or erratic, fast or slow, strong or weak or may feel like a missed or extra beat that can occur during exercise, www.acsm-csmr.org

emotional situations, and stress and sometimes without any provoking factors (5,16). The figure shows common cardiac and noncardiac causes of palpitations (13,20). The variability of clinical outcomes of palpitations, ranging from a single benign event to the first sign of a potentially life-threatening problem, makes the evaluation of palpitations a challenging process (17). In two clinical studies, a clinically significant arrhythmia was diagnosed in one-fifth of patients presenting with palpitations (8,24) while a lower prevalence of pathology is found in healthy young athletes (13). The prevalence of palpitations in athletes varies widely depending on age and type of sports. The fourth edition PPE monograph inquires exclusively about palpitations during exercise, and query regarding exertional palpitations is now included in the 2014 AHA elements for preparticipation evaluation (15). When the answer is positive for exertional palpitations, a careful history examination should be conducted to determine whether further evaluation is needed and which diagnostic testing should be ordered. The critical questions are listed in Table 1. 1) Analysis of the complaint This should start by an open-ended question without prompting but noted in the record. Asking the athlete to tap the rhythm of their palpitations or to choose from rhythms tapped by the physician can be helpful (Table 2) (25). Palpitations associated with syncope or presyncope may signify a clinical significant arrhythmia such as supraventricular or ventricular tachycardia (VT) in athletes (14,29). Palpitations while sitting quietly or lying in bed that last for brief periods and felt as a single skipped beat or a sensation of stopping of the heartbeats and then restarting suggest premature atrial or ventricular extrasystoles (1,29). Palpitations are not likely to be due to a significant cardiac arrhythmia when the onset and termination are gradual (20). For example, a sudden onset and abrupt termination of an irregularly irregular heartbeat reinforced by atrial fibrillation (AF) type tapping out along with weakness and fatigue suggest AF. However, sudden onset of fast regular palpitations that continue after stopping exercise broken by positional change or vagal maneuver that can be associated with polyuria, pounding sensation in the neck, shirt flapping, and/or visible cannon A waves in the neck ‘‘frog sign’’ suggests a supraventricular reentry tachyarrhythmia particularly atrioventricular reentrant tachycardia (AVRT) and atrioventricular nodal reentrant tachycardia (AVNRT) (2,7,11,20). Patients with regular palpitations were found to be twice more likely to have a ‘‘significant’’ cardiac arrhythmia as a cause for their palpitations (24). When palpitations are felt as strong or powerful contractions, but regular and persistent, the physician should think of a systemic cause, such as a high stroke volume or a structural heart disease, such as aortic regurgitation (20). Frequent extra beats, some with super hard beats, some running in a row or associated with weakness or collapse, could be premature ventricular contractions (PVC) or VT. Current Sports Medicine Reports


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be included, as they can be the sole cause of palpitations or may exacerbate any underlying cardiac pathology. Increased caffeine intake is usually associated with headache, tremor, and insomnia in addition to palpitations (23). Exposure to medications, supplements, illicit drugs, or performance-enhancing agents (PEA) should be questioned. The World Anti-Doping Agency Web site (http://www.list. wada-ama.org/prohibited-all-times/prohibited-substances/) has a complete sports-specific listing of approved and nonapproved drugs. Also, there is a list of medications affecting QT duration at http://www.sads.org.uk/drugs_to_avoid.htm. In females, benign premature atrial contractions (PAC) have been linked to the use of oral contraceptive pills or the fluctuating levels of estrogen during the menstrual cycle (13). Other drugs like beta-agonists, thyroid hormones, and weight reduction drugs can cause palpitations. A statistical difference was found between using marijuana and/or cocaine and the occurrence of palpitations than nonusers (18).

Figure: Etiology of palpitations.

Even though palpitations can trigger a panic attack, usually the onset of a panic attack precedes the palpitations and is associated with numbness around the mouth and hand spasms. Often, individuals with panic attacks have learned to stop them by breathing into a paper bag or can be told to try this maneuver and will find it effective in stopping their palpitations.

Physical Examination Physical examination does not add much if the athlete is in normal rhythm, but auscultation of the heart is important to detect any murmurs of structural heart diseases. You may be fortunate enough to detect some irregularity suggestive of AF or just simple PVC or PAC that could immediately be confirmed with a simple single lead ECG recorder, even one working through your smartphone (AliveCori). The physician should look for any signs of anemia, thyroid diseases, hyperdynamic state, anxiety, or fever.

2) Obtaining a family history This is considered the most important component in the history taking of athletes complaining of palpitations. The physician should inquire not only for a history of sudden death but also for other deaths that may have been a manifestation of arrhythmia, such as the sudden infant death syndrome, drowning, near drowning, and unexplained car accident, as these might have been due to syncope (3,22). This question should consider only first-degree family members (G40 years old) (19,28). A family history of syncope and sudden cardiac death (SCD), palpitations, supine syncope, and syncope associated with exercise and emotional stress should alert physicians to the high risk for Wolff-Parkinson-White (WPW) syndrome and Long QT syndrome (LQTS) in this individual. Accordingly, the physician should obtain an ECG paying attention to the WPW pattern or the QT interval respectively (6).

Table 1.

3) Identification of noncardiac causes The next step would be the identification of any existent noncardiac causes and acting accordingly. Emotions can initiate many significant arrhythmias (10), while panic attacks are usually accompanied by tachycardia (4). However, a combination of cardiac and psychiatric causes can coexist (9), so it remains very important to rule out any underlying cardiac etiology of palpitations (9,26,27). Anxiety-related palpitations are usually preceded by unspecific symptoms, such as tingling in the hands and face, a lump in the throat, mental confusion, agitation, atypical chest pains, and sighing dyspnea (20). Direct questioning about alcohol consumption and caffeine intake (including sodas and energy drinks) (21) should

7) Have you noticed any associated symptoms? Visual? Lightheadedness? Falling? Loss of consciousness?

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Volume 14 & Number 4 & July/August 2015

Key history questions.

Analysis of the complaint 1) Tell me more about your "palpitations." Could you describe them for me? 2) When they occur, do the heartbeats feel regular or irregular? Fast or slow? Weak or strong? 3) How often do you get palpitations? How long does each episode last? What brings them on? 4) Do the palpitations start and stop gradually? Suddenly? 5) Is there anything that can stop these palpitations when they occur? Have you noticed anything that brings them back? 6) When usually do they occur? During exercise? After effort? At rest?

Family history 8) Is there any family history of cardiac diseases, arrhythmias, drowning, unexplained accidental deaths, or SCD? Identification of noncardiac cause 9) Do you feel that you are nervous or anxious by nature? 10) Any history prior to the palpitations of alcohol consumption or caffeine intake? Illicit drugs? 11) Are you currently taking any antianxiety or other medications? Supplements? PEA? Weight-reducing agents?



Table 2. Clinical characteristics of different cardiac arrhythmia.

Type of Arrhythmia

Atrial Tachycardia

Atrial Flutter

AF

AVRT, AVNRT

VT

Premature Atrial or Ventricular Extra Systoles

Onset and termination

Sudden

Sudden

Sudden

Sudden

Sudden

Heart rhythm

Mostly regular

Irregular

Regular

Regular

Irregular

Increased

Variable

Increased

Increased

Physical effort, cooling down, after meal, alcohol intake

Physical effort, changes in posture

Physical effort

Transitory reduction in heart rate

Sudden interruption

No effect

Heart rate Triggers

Vagal maneuvers

Transitory reduction in heart rate

Patient description

Beating wings/ rapid fluctuations

Associated symptoms

Polyuria, weakness, fatigue

Physical examination

Irregularly irregular pulse, pulse deficit or variable first heart sound intensity

Rest

Missing/skipping a beat, sinking of the heart, or heart seems to stop and then start Polyuria, frog sign (witnessed neck pulsations), shirt flapping

Signs/symptoms of hemodynamic impairment, shirt flapping Cannon A waves on jugular venous pressure

Comment

PAC: usually benign; if numerous/ repetitive causing disabling symptoms, treat BB/CCB

References used (3,4,12). AVRT, Atrioventricular reentrant tachycardia; AVNRT, Atrioventricular nodal reentrant tachycardia. BB, beta-blockers; CCB, calcium channel blockers.

Diagnostic Evaluation 12-lead ECG The first step could be a standard ECG with a rhythm strip, but the real challenge is to capture a recording of the cardiac rhythm while experiencing palpitations. ECG monitor If the palpitations remain unexplained, a prolonged ECG monitoring is needed to find any underlying arrhythmias. The usual clinical options are available including Holter monitoring and event recorders, but these require carrying a device and using cables to connect to electrodes. These devices can interfere with an athlete’s usual exercise program, so we now prefer to use adhesive patches that have the electrodes and ECG electronics combined and do not require cables (Zio Patchi). We have even used these for swimmers by covering them with water-proof bandages. Exercise test An exercise test can be helpful to evaluate whether the arrhythmia is brought out by exercise or occur in the recovery period. Catecholaminergic polymorphic VT can be brought out by exercise and QT prolongation associated with LQTS. www.acsm-csmr.org

Echocardiography After the cause of the palpitations is determined to be pathophysiological but not a conduction problem, an echocardiogram can be helpful to see whether a cardiomyopathy or other structural problem is the cause. Referral Once the palpitations are diagnosed as an arrhythmia, referral to a cardiologist or electrophysiologist is appropriate. Otherwise, reassurance and/or evaluation of psychological issues are the next steps. References 1. Abbott AV. Diagnostic approach to palpitations. Am. Fam. Physician. 2005; 71:743Y50. 2. Abe H, Nagatomo T, Kobayashi H, et al. Neurohumoral and hemodynamic mechanisms of diuresis during atrioventricular nodal reentrant tachycardia. Pacing Clin. Electrophysiol. 1997; 20:2783Y8. 3. Arnestad M, Crotti L, Rognum TO, et al. Prevalence of long-QT syndrome gene variants in sudden infant death syndrome. Circulation. 2007; 115:361Y7. 4. Barsky AJ, Cleary PD, Sarnie MK, Ruskin JN. Panic disorder, palpitations, and the awareness of cardiac activity. J. Nerv. Ment. Dis. 1994; 182:63Y71. 5. Brugada P, Gu¨rsoy S, Brugada J, Andries E. Investigation of palpitations. Lancet. 1993; 341:1254Y8.



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6. Colman N, Bakker A, Linzer M, et al. Value of history-taking in syncope patients: in whom to suspect long QT syndrome? Europace. 2009; 11:937Y43. 7. Gu¨rsoy S, Steurer G, Brugada J, et al. Brief report: the hemodynamic mechanism of pounding in the neck in atrioventricular nodal reentrant tachycardia. N. Engl. J. Med. 1992; 327:772Y4. 8. Hoefman E, Boer KR, van Weert HC, et al. Predictive value of history taking and physical examination in diagnosing arrhythmias in general practice. Fam. Pract. 2007; 24:636Y41. 9. Jamshed N, Dubin J, Eldadah Z. Emergency management of palpitations in the elderly: epidemiology, diagnostic approaches, and therapeutic options. Clin. Geriatr. Med. 2013; 29:205Y30. 10. Lampert R, Joska T, Burg MM, et al. Emotional and physical precipitants of ventricular arrhythmia. Circulation. 2002; 106:1800Y5. 11. Laurent G, Leong-Poi H, Mangat I, et al. Influence of ventriculoatrial timing on hemodynamics and symptoms during supraventricular tachycardia. J. Cardiovasc. Electrophysiol. 2009; 20:176Y81. 12. Lawless CE, Asplund C, Courson R, et al. Protecting the heart of the American athlete. J. Am. Coll. Cardiol. 2014; 64:2146Y71. 13. Lawless CE, Briner W. Palpitations in athletes. Sports Med. 2008; 38:687Y702. 14. Leitch JW, Klein GJ, Yee R, et al. Syncope associated with supraventricular tachycardia. An expression of tachycardia rate or vasomotor response? Circulation. 1992; 85:1064Y71. 15. Maron BJ, Friedman RA, Kligfield P, et al. Assessment of the 12-Lead ECG as a screening test for detection of cardiovascular disease in healthy general populations of young people (12Y25 years of age): a scientific statement from the American Heart Association and the American College of Cardiology. Circulation. 2014; 130:1303Y34. 16. Mayou R, Sprigings D, Birkhead J, Price J. Characteristics of patients presenting to a cardiac clinic with palpitation. QJM. 2003; 96:115Y23. 17. Misiri J, Candler S, Kusumoto FM. Evaluation of syncope and palpitations in women. J. Womens Health (Larchmt). 2011; 20:1505Y15. 18. Petronis KR, Anthony JC. An epidemiologic investigation of marijuana- and cocaine-related palpitations. Drug Alcohol Depend. 1989; 23:219Y26. 19. Ranthe MF, Winkel BG, Andersen EW, et al. Risk of cardiovascular disease in family members of young sudden cardiac death victims. Eur. Heart J. 2013; 34:503Y11. 20. Raviele A, Giada F, Bergfeldt L, et al. Management of patients with palpitations: a position paper from the European Heart Rhythm Association. Europace. 2011; 13:920Y34. 21. Reissig CJ, Strain EC, Griffiths RR. Caffeinated energy drinksVa growing problem. Drug Alcohol Depend. 2009; 99:1Y10. 22. Roden DM. Clinical practice. Long-QTsyndrome. N. Engl. J. Med. 2008; 358:169Y76. 23. Shirlow MJ, Mathers CD. A study of caffeine consumption and symptoms; indigestion, palpitations, tremor, headache and insomnia. Int. J. Epidemiol. 1985; 14:239Y48. 24. Summerton N, Mann S, Rigby A, et al. New-onset palpitations in general practice: assessing the discriminant value of items within the clinical history. Fam. Pract. 2001; 18:383Y92. 25. Thavendiranathan P, Bagai A, Khoo C, et al. Does this patient with palpitations have a cardiac arrhythmia? JAMA. 2009; 302:2135Y43. 26. Weber BE, Kapoor WN. Evaluation and outcomes of patients with palpitations. Am. J. Med. 1996; 100:138Y48. 27. Wexler RK, Pleister A, Raman S. Outpatient approach to palpitations. Am. Fam. Physician. 2011; 84:63Y9. 28. Wong LC, Roses-Noguer F, Till JA, Behr ER. Cardiac evaluation of pediatric relatives in sudden arrhythmic death syndrome: a 2-center experience. Circ. Arrhythm. Electrophysiol. 2014; 7:800Y6. 29. Zimetbaum P, Josephson ME. Evaluation of patients with palpitations. N. Engl. J. Med. 1998; 338:1369Y73.

Sudden Death in the Family from Personal History Section Ricardo Stein, MD, ScD and Anderson Donelli da Silveira, MD

Personal History: Has anyone in your family younger than 50 years of age died suddenly or unexpectedly from heart disease? Young athletes should be instructed to talk to their parents or their surrogates to obtain this information. To determine 336


the importance of a positive answer to this question, the following questions can be helpful. 1) FOLLOW-UP QUESTIONING The goal of the investigation was to identify athletes at higher risk for inherited cardiovascular disorders. These include the cardiomyopathies, channelopathies, and primary arrhythmic disturbances. Many of these diseases carry autosomal-dominant inheritance patterns, so a family history of these diseases can focus the evaluation. A positive response to sudden cardiac death from heart disease in a family member G50 years old should trigger three vital questions that provide valuable information for risk assessment and stratification for the athlete, as follows:

a) the exact relation of the family member who died; b) the age at which the death occurred, especially under age 35; and c) the cardiovascular disease that caused the death. 2) FAMILY HISTORY OF SDC G35 YEARS OLD Since SCD in adults over 35 years old is likely due to atherosclerotic disease, the specificity for detecting disease will be highest for athletes with a family history of SCD G35 years old. The cause of death should be pursued for the 35- to 50-year relatives, as CAD will have limited impact on exercise recommendations for younger athletes, and occasionally a person over age 35 will succumb to channelopathies or aberrant coronary arteries. A large controlled epidemiologic study from Denmark provides evidence that firstdegree family members of young SCD victims are at highest risk (1,2). Over an 11-year follow-up period, the risk was highest among first-degree relatives, where cardiomyopathy was 18-fold higher and ventricular arrhythmias were 19-fold greater than of those without a family history of early SCD. Results from this study suggest that athletes with a family history of sudden cardiac death G35 years old should undergo a more comprehensive cardiac evaluation with an electrocardiography (ECG) and referral to cardiology for further testing. 3) SIGNS, SYMPTOMS, AND CIRCUMSTANCES SURROUNDING THE FAMILY MEMBER’S DEATH Many athletes may not know the circumstances for their family member’s sudden death. However, information such as the signs, symptoms, or circumstances of death can be invaluable. For example, information surrounding the time of day, location, and association with exercise may provide information surrounding an unexplained accident. The most common variant of LQTS, type 1, is associated with arrhythmias in water. Thus, it is essential to have a high index of suspicion for individuals with a family history of unexplained death while swimming. LQTS individuals may also experience arrhythmias during periods of intense emotion or loud startling noises. 4) DEVELOPING A FAMILY TREE A family tree can be synthesized when more time can be spent with the athlete. However, many are unaware of the circumstances or conditions of their family members. In Cardiovascular Preparticipation Evaluation


6. Colman N, Bakker A, Linzer M, et al. Value of history-taking in syncope patients: in whom to suspect long QT syndrome? Europace. 2009; 11:937Y43. 7. Gu¨rsoy S, Steurer G, Brugada J, et al. Brief report: the hemodynamic mechanism of pounding in the neck in atrioventricular nodal reentrant tachycardia. N. Engl. J. Med. 1992; 327:772Y4. 8. Hoefman E, Boer KR, van Weert HC, et al. Predictive value of history taking and physical examination in diagnosing arrhythmias in general practice. Fam. Pract. 2007; 24:636Y41. 9. Jamshed N, Dubin J, Eldadah Z. Emergency management of palpitations in the elderly: epidemiology, diagnostic approaches, and therapeutic options. Clin. Geriatr. Med. 2013; 29:205Y30. 10. Lampert R, Joska T, Burg MM, et al. Emotional and physical precipitants of ventricular arrhythmia. Circulation. 2002; 106:1800Y5. 11. Laurent G, Leong-Poi H, Mangat I, et al. Influence of ventriculoatrial timing on hemodynamics and symptoms during supraventricular tachycardia. J. Cardiovasc. Electrophysiol. 2009; 20:176Y81. 12. Lawless CE, Asplund C, Courson R, et al. Protecting the heart of the American athlete. J. Am. Coll. Cardiol. 2014; 64:2146Y71. 13. Lawless CE, Briner W. Palpitations in athletes. Sports Med. 2008; 38:687Y702. 14. Leitch JW, Klein GJ, Yee R, et al. Syncope associated with supraventricular tachycardia. An expression of tachycardia rate or vasomotor response? Circulation. 1992; 85:1064Y71. 15. Maron BJ, Friedman RA, Kligfield P, et al. Assessment of the 12-Lead ECG as a screening test for detection of cardiovascular disease in healthy general populations of young people (12Y25 years of age): a scientific statement from the American Heart Association and the American College of Cardiology. Circulation. 2014; 130:1303Y34. 16. Mayou R, Sprigings D, Birkhead J, Price J. Characteristics of patients presenting to a cardiac clinic with palpitation. QJM. 2003; 96:115Y23. 17. Misiri J, Candler S, Kusumoto FM. Evaluation of syncope and palpitations in women. J. Womens Health (Larchmt). 2011; 20:1505Y15. 18. Petronis KR, Anthony JC. An epidemiologic investigation of marijuana- and cocaine-related palpitations. Drug Alcohol Depend. 1989; 23:219Y26. 19. Ranthe MF, Winkel BG, Andersen EW, et al. Risk of cardiovascular disease in family members of young sudden cardiac death victims. Eur. Heart J. 2013; 34:503Y11. 20. Raviele A, Giada F, Bergfeldt L, et al. Management of patients with palpitations: a position paper from the European Heart Rhythm Association. Europace. 2011; 13:920Y34. 21. Reissig CJ, Strain EC, Griffiths RR. Caffeinated energy drinksVa growing problem. Drug Alcohol Depend. 2009; 99:1Y10. 22. Roden DM. Clinical practice. Long-QTsyndrome. N. Engl. J. Med. 2008; 358:169Y76. 23. Shirlow MJ, Mathers CD. A study of caffeine consumption and symptoms; indigestion, palpitations, tremor, headache and insomnia. Int. J. Epidemiol. 1985; 14:239Y48. 24. Summerton N, Mann S, Rigby A, et al. New-onset palpitations in general practice: assessing the discriminant value of items within the clinical history. Fam. Pract. 2001; 18:383Y92. 25. Thavendiranathan P, Bagai A, Khoo C, et al. Does this patient with palpitations have a cardiac arrhythmia? JAMA. 2009; 302:2135Y43. 26. Weber BE, Kapoor WN. Evaluation and outcomes of patients with palpitations. Am. J. Med. 1996; 100:138Y48. 27. Wexler RK, Pleister A, Raman S. Outpatient approach to palpitations. Am. Fam. Physician. 2011; 84:63Y9. 28. Wong LC, Roses-Noguer F, Till JA, Behr ER. Cardiac evaluation of pediatric relatives in sudden arrhythmic death syndrome: a 2-center experience. Circ. Arrhythm. Electrophysiol. 2014; 7:800Y6. 29. Zimetbaum P, Josephson ME. Evaluation of patients with palpitations. N. Engl. J. Med. 1998; 338:1369Y73.

Sudden Death in the Family from Personal History Section Ricardo Stein, MD, ScD and Anderson Donelli da Silveira, MD

Personal History: Has anyone in your family younger than 50 years of age died suddenly or unexpectedly from heart disease? Young athletes should be instructed to talk to their parents or their surrogates to obtain this information. To determine 336


the importance of a positive answer to this question, the following questions can be helpful. 1) FOLLOW-UP QUESTIONING The goal of the investigation was to identify athletes at higher risk for inherited cardiovascular disorders. These include the cardiomyopathies, channelopathies, and primary arrhythmic disturbances. Many of these diseases carry autosomal-dominant inheritance patterns, so a family history of these diseases can focus the evaluation. A positive response to sudden cardiac death from heart disease in a family member G50 years old should trigger three vital questions that provide valuable information for risk assessment and stratification for the athlete, as follows:

a) the exact relation of the family member who died; b) the age at which the death occurred, especially under age 35; and c) the cardiovascular disease that caused the death. 2) FAMILY HISTORY OF SDC G35 YEARS OLD Since SCD in adults over 35 years old is likely due to atherosclerotic disease, the specificity for detecting disease will be highest for athletes with a family history of SCD G35 years old. The cause of death should be pursued for the 35- to 50-year relatives, as CAD will have limited impact on exercise recommendations for younger athletes, and occasionally a person over age 35 will succumb to channelopathies or aberrant coronary arteries. A large controlled epidemiologic study from Denmark provides evidence that firstdegree family members of young SCD victims are at highest risk (1,2). Over an 11-year follow-up period, the risk was highest among first-degree relatives, where cardiomyopathy was 18-fold higher and ventricular arrhythmias were 19-fold greater than of those without a family history of early SCD. Results from this study suggest that athletes with a family history of sudden cardiac death G35 years old should undergo a more comprehensive cardiac evaluation with an electrocardiography (ECG) and referral to cardiology for further testing. 3) SIGNS, SYMPTOMS, AND CIRCUMSTANCES SURROUNDING THE FAMILY MEMBER’S DEATH Many athletes may not know the circumstances for their family member’s sudden death. However, information such as the signs, symptoms, or circumstances of death can be invaluable. For example, information surrounding the time of day, location, and association with exercise may provide information surrounding an unexplained accident. The most common variant of LQTS, type 1, is associated with arrhythmias in water. Thus, it is essential to have a high index of suspicion for individuals with a family history of unexplained death while swimming. LQTS individuals may also experience arrhythmias during periods of intense emotion or loud startling noises. 4) DEVELOPING A FAMILY TREE A family tree can be synthesized when more time can be spent with the athlete. However, many are unaware of the circumstances or conditions of their family members. In Cardiovascular Preparticipation Evaluation


these cases, it is important to engage in conversation with parents or guardians who may elicit essential details. Guidelines for Developing a Thorough Family Tree a) Record as many family members as possible. b) Highlight the presence of sudden death or heart disease, including diagnosis. c) Indicate the age of affected individuals. d) Determine the circumstances of the death (association with exercise or during rest, prior symptoms, etc.). e) Identify potential coronary risk factors in affected individuals. f) Note pharmacological treatment, procedures, and/or response to defibrillation. KEY POINTS a) A family history of cardiomyopathy, channelopathy, or conduction disturbance necessitates further questioning and investigation. b) Sudden cardiac death in relatives G35 years old is worrisome for a potentially inherited cardiovascular condition. Additional testing should include, at minimum, an ECG and cardiology consultation.

the age of 4 during sleep. The athlete’s brother was asymptomatic, but an ECG demonstrated a QT interval of 511 ms. Genetic testing of the family revealed several family members with the KCNQ1 mutation. Key point:

&A

thorough family history examination is needed during the preparticipation examination. Athletes with a family history of unexplained death at a young age (especially G35 years old) require further evaluation with an ECG and referral to cardiology

References 1. Asif IM, Drezner JA. Detecting occult cardiac disease in athletes: history that makes a difference. Br. J. Sports Med. 2013; 47:669. 2. Ranthe MF, Winkel BG, Andersen EW, et al. Risk of cardiovascular disease in family members of young sudden cardiac death victims. Eur. Heart J. 2013; 34:503Y11.

Family History of Cardiovascular Disability from Family History Section Marco Perez, MD

CASE EXAMPLES Young Soccer Player with a Father Who Died Unexpectedly A 12 year-old Italian soccer player underwent a PPE to enter the sports academy as part of the national development team. Upon investigation of his family history, it was noted that his father (asymptomatic) died suddenly at age 34. In addition, the father’s uncle had progressive symptoms of heart failure since the second decade of life. The athlete himself was asymptomatic, and his physical examination result was normal. A cardiac magnetic resonance imaging was ordered, and arrhythmogenic right ventricular dysplasia/cardiomyopathy was diagnosed. Key points & ARVC is difficult to identify with imaging, so it is helpful to appreciate that 30% of diagnosed patients have a relative with this diagnosis. & Further testing is warranted in athletes with a family history of SCD G35 years old. Additional workup will be guided by the differential diagnosis created after history and physical examination and ECG. Swimmer with Long QT Syndrome A 17-year-old Norwegian long-distance swimmer had a cardiac arrest during competition. The initial rhythm was ventricular fibrillation, and a defibrillator was used immediately for resuscitation. While in the intensive care unit, ECG revealed a corrected QT interval of 475 ms with a broad T wave. He was on no prior medication or supplements, and his laboratory results, including electrolytes, were within normal limits. Treatment consisted of beta-blockers and an implantable cardioverter defibrillator (ICD). While in recovery, a meticulous family history was taken. His parents were both healthy with normal QT interval on ECG. However, he had an uncle who died unexpectedly at www.acsm-csmr.org

Question 7: Has anyone in your family younger than 50 years of age been disabled from heart disease or had cardiac treatments including surgery? The purpose of this question is to probe the athlete for family history of an inherited cardiovascular (CV) illness that could result in sudden death. Patients and athletes are often poor family historians and may not be able to name a condition that runs in the family. However, they are often exposed to and more easily recall disabling features of inherited CV disease. Questions that probe indirectly for these conditions may uncover underlying familial illness. To determine the importance of a positive answer to this question, the following questions should be asked:

1) Can you name the heart disease that caused your family member’s disability? While this question is designed to assess for the presence of specific inherited CV disorders in the family, the information is likely to be more accurate if the athlete can name the condition without prompting. The most common inherited disabling CV conditions are the familial cardiomyopathies (hypertrophic or dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and left ventricular noncompaction), the channelopathies (long QT syndrome, Brugada syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia), premature coronary disease from familial hyperlipidemia, and Marfan syndrome. Other cardiac conditions that could leave a young family member disabled but that do not have a strong heritable component include rheumatic heart disease, myocarditis, sarcoidosis, and anomalous coronary artery anatomy. A family history of these conditions may not necessitate further investigation. If the athlete cannot name the disabling condition, then the following questions may help determine Current Sports Medicine Reports


337

these cases, it is important to engage in conversation with parents or guardians who may elicit essential details. Guidelines for Developing a Thorough Family Tree a) Record as many family members as possible. b) Highlight the presence of sudden death or heart disease, including diagnosis. c) Indicate the age of affected individuals. d) Determine the circumstances of the death (association with exercise or during rest, prior symptoms, etc.). e) Identify potential coronary risk factors in affected individuals. f) Note pharmacological treatment, procedures, and/or response to defibrillation. KEY POINTS a) A family history of cardiomyopathy, channelopathy, or conduction disturbance necessitates further questioning and investigation. b) Sudden cardiac death in relatives G35 years old is worrisome for a potentially inherited cardiovascular condition. Additional testing should include, at minimum, an ECG and cardiology consultation.

the age of 4 during sleep. The athlete’s brother was asymptomatic, but an ECG demonstrated a QT interval of 511 ms. Genetic testing of the family revealed several family members with the KCNQ1 mutation. Key point:

&A

thorough family history examination is needed during the preparticipation examination. Athletes with a family history of unexplained death at a young age (especially G35 years old) require further evaluation with an ECG and referral to cardiology

References 1. Asif IM, Drezner JA. Detecting occult cardiac disease in athletes: history that makes a difference. Br. J. Sports Med. 2013; 47:669. 2. Ranthe MF, Winkel BG, Andersen EW, et al. Risk of cardiovascular disease in family members of young sudden cardiac death victims. Eur. Heart J. 2013; 34:503Y11.

Family History of Cardiovascular Disability from Family History Section Marco Perez, MD

CASE EXAMPLES Young Soccer Player with a Father Who Died Unexpectedly A 12 year-old Italian soccer player underwent a PPE to enter the sports academy as part of the national development team. Upon investigation of his family history, it was noted that his father (asymptomatic) died suddenly at age 34. In addition, the father’s uncle had progressive symptoms of heart failure since the second decade of life. The athlete himself was asymptomatic, and his physical examination result was normal. A cardiac magnetic resonance imaging was ordered, and arrhythmogenic right ventricular dysplasia/cardiomyopathy was diagnosed. Key points & ARVC is difficult to identify with imaging, so it is helpful to appreciate that 30% of diagnosed patients have a relative with this diagnosis. & Further testing is warranted in athletes with a family history of SCD G35 years old. Additional workup will be guided by the differential diagnosis created after history and physical examination and ECG. Swimmer with Long QT Syndrome A 17-year-old Norwegian long-distance swimmer had a cardiac arrest during competition. The initial rhythm was ventricular fibrillation, and a defibrillator was used immediately for resuscitation. While in the intensive care unit, ECG revealed a corrected QT interval of 475 ms with a broad T wave. He was on no prior medication or supplements, and his laboratory results, including electrolytes, were within normal limits. Treatment consisted of beta-blockers and an implantable cardioverter defibrillator (ICD). While in recovery, a meticulous family history was taken. His parents were both healthy with normal QT interval on ECG. However, he had an uncle who died unexpectedly at www.acsm-csmr.org

Question 7: Has anyone in your family younger than 50 years of age been disabled from heart disease or had cardiac treatments including surgery? The purpose of this question is to probe the athlete for family history of an inherited cardiovascular (CV) illness that could result in sudden death. Patients and athletes are often poor family historians and may not be able to name a condition that runs in the family. However, they are often exposed to and more easily recall disabling features of inherited CV disease. Questions that probe indirectly for these conditions may uncover underlying familial illness. To determine the importance of a positive answer to this question, the following questions should be asked:

1) Can you name the heart disease that caused your family member’s disability? While this question is designed to assess for the presence of specific inherited CV disorders in the family, the information is likely to be more accurate if the athlete can name the condition without prompting. The most common inherited disabling CV conditions are the familial cardiomyopathies (hypertrophic or dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and left ventricular noncompaction), the channelopathies (long QT syndrome, Brugada syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia), premature coronary disease from familial hyperlipidemia, and Marfan syndrome. Other cardiac conditions that could leave a young family member disabled but that do not have a strong heritable component include rheumatic heart disease, myocarditis, sarcoidosis, and anomalous coronary artery anatomy. A family history of these conditions may not necessitate further investigation. If the athlete cannot name the disabling condition, then the following questions may help determine Current Sports Medicine Reports


337

the likelihood that a positive response to this question is due to a familial inherited condition.

2) What treatments or surgeries has your family member had? A family member may be more likely to remember dramatic clinical consequences of the condition rather than the name of the condition itself. A family member who had an intracardiac defibrillator (ICD) placed before the age of 50 (especially before age 35), for example, is an important flag that they either had an aborted arrest or were deemed at high risk for sudden death due to an inherited channelopathy or cardiomyopathy. Many patients confuse the term ‘‘defibrillator’’ with ‘‘pacemaker’’; because of this, an athlete who reports a family member had a ‘‘pacemaker’’ placed at an early age should be at risk for an inherited CV disease. Another easily remembered dramatic intervention that signals a possible inherited condition is heart transplant, which if done before the age of 50 may signal an inherited or acquired cardiomyopathy. A family history of open heart surgery (coronary bypass surgery, valvular surgery, aortic surgery) at an early age also may be a marker for premature coronary disease, Marfan syndrome, or congenital heart disease and also should warrant further evaluation. Likewise, early percutaneous coronary intervention would be a marker of premature coronary disease; however, a young athlete may find it difficult to recognize and report when this has been done in a family member. There are many other treatments that could be administered to young family members without necessary concern for inherited CV conditions. Catheter-based ablations for supraventricular tachycardias or medical therapy for hypertension or hyperlipidemia are examples of treatments that would not necessarily raise concern.

3) How severely is your family member disabled? The term ‘‘disability’’ could be interpreted in different ways by the athlete being questioned. Asking the athlete to describe the disability can help distinguish disabilities from cardiopulmonary as opposed to musculoskeletal disorders. One of the important characteristics to determine degree of disability includes assessment of whether the disability interferes with the family member’s ability to work, exercise, go to school, or perform normal daily activities. The inherited cardiomyopathies are the conditions most likely to result in early major cardiopulmonary disability. CASE EXAMPLES Case 1 A 15-year-old male high school cross-country competitor underwent PPE screening. He was otherwise healthy with no chest pain, syncope, shortness of breath, or palpitations and normal physical examination results. He reported his mother had a ‘‘pacemaker’’ placed, but he did not know what the reason for this was. Upon further investigation into the family history, it was discovered that his mother was diagnosed with long QT syndrome and had undergone implantation of an ICD for recurrent syncope and a pathogenic KCNQ1 variant genotype consistent with long QT subtype 1. The athlete had an 338


ECG showing a normal QT interval, but genetic testing revealed that he was a carrier of the KCNQ1 variant. Despite counseling regarding the higher risk of sudden death, he and his family chose to continue sports participation but with frequent ECG screening and AICD present at all practices and competitions. Case 2 A 19-year-old female college rower reported for PPE screening. She denied any chest pain, syncope, shortness of breath or palpitations. Her physical examination was unremarkable. She reported that her maternal grandmother had ‘‘heart surgery’’ when she was younger, but she was not sure what type, and that her grandmother could never participate in many of the family activities. The athlete had an ECG that was unremarkable except for a borderline prolonged QTc at 468ms. Upon further investigation into the family history, it was found that her grandmother had undergone myomectomy for hypertrophic cardiomyopathy. Genetic testing had revealed a causative variant in MYH7. The athlete had an echocardiogram which was unremarkable, and genetic testing that showed she was not an MYH7 variant carrier. The athlete continued to row but will have yearly ECG because of the borderline QT prolongation.

Family History of Hypertrophic or Dilated Cardiomyopathy from Family History Section Frederick Dewey, MD

Question 8a. Does anyone in your family have any of the following: hypertrophic cardiomyopathy (HCM) or dilated cardiomyopathy (DCM)? The purpose of this question is to probe specifically for the more common familial cardiomyopathies that can lead to sudden death on the playing field. Familial HCM exists with an estimated prevalence of 1/500 in the general population, and many affected patients are asymptomatic at the time of diagnosis (1,2). Familial DCM accounts for an estimated 20% to 50% of cases of ‘‘idiopathic’’ DCM. These conditions have highly variable penetrance (that is, the proportion of genetically at-risk patients with clinical disease), age of onset, and clinical presentations (3). Both diseases can be mimicked by acquired forms of heart disease such as hypertension and coronary artery disease in older athletes, and adaptive training-related changes may be difficult to distinguish from pathologic primary cardiomyopathy. Because these conditions are rare, many screening physicians are unfamiliar with the initial presentation and diagnostic workup, and the true diagnosis may be elusive or uncertain in some family members. For these reasons, it can be challenging to assess the true risk for familial HCM or DCM-related cardiovascular events. A series of follow-up questions can clarify family history and uncover characteristic clinical findings, suggesting a true genetic risk for HCM or DCM. Cardiovascular Preparticipation Evaluation


the likelihood that a positive response to this question is due to a familial inherited condition.

2) What treatments or surgeries has your family member had? A family member may be more likely to remember dramatic clinical consequences of the condition rather than the name of the condition itself. A family member who had an intracardiac defibrillator (ICD) placed before the age of 50 (especially before age 35), for example, is an important flag that they either had an aborted arrest or were deemed at high risk for sudden death due to an inherited channelopathy or cardiomyopathy. Many patients confuse the term ‘‘defibrillator’’ with ‘‘pacemaker’’; because of this, an athlete who reports a family member had a ‘‘pacemaker’’ placed at an early age should be at risk for an inherited CV disease. Another easily remembered dramatic intervention that signals a possible inherited condition is heart transplant, which if done before the age of 50 may signal an inherited or acquired cardiomyopathy. A family history of open heart surgery (coronary bypass surgery, valvular surgery, aortic surgery) at an early age also may be a marker for premature coronary disease, Marfan syndrome, or congenital heart disease and also should warrant further evaluation. Likewise, early percutaneous coronary intervention would be a marker of premature coronary disease; however, a young athlete may find it difficult to recognize and report when this has been done in a family member. There are many other treatments that could be administered to young family members without necessary concern for inherited CV conditions. Catheter-based ablations for supraventricular tachycardias or medical therapy for hypertension or hyperlipidemia are examples of treatments that would not necessarily raise concern.

3) How severely is your family member disabled? The term ‘‘disability’’ could be interpreted in different ways by the athlete being questioned. Asking the athlete to describe the disability can help distinguish disabilities from cardiopulmonary as opposed to musculoskeletal disorders. One of the important characteristics to determine degree of disability includes assessment of whether the disability interferes with the family member’s ability to work, exercise, go to school, or perform normal daily activities. The inherited cardiomyopathies are the conditions most likely to result in early major cardiopulmonary disability. CASE EXAMPLES Case 1 A 15-year-old male high school cross-country competitor underwent PPE screening. He was otherwise healthy with no chest pain, syncope, shortness of breath, or palpitations and normal physical examination results. He reported his mother had a ‘‘pacemaker’’ placed, but he did not know what the reason for this was. Upon further investigation into the family history, it was discovered that his mother was diagnosed with long QT syndrome and had undergone implantation of an ICD for recurrent syncope and a pathogenic KCNQ1 variant genotype consistent with long QT subtype 1. The athlete had an 338


ECG showing a normal QT interval, but genetic testing revealed that he was a carrier of the KCNQ1 variant. Despite counseling regarding the higher risk of sudden death, he and his family chose to continue sports participation but with frequent ECG screening and AICD present at all practices and competitions. Case 2 A 19-year-old female college rower reported for PPE screening. She denied any chest pain, syncope, shortness of breath or palpitations. Her physical examination was unremarkable. She reported that her maternal grandmother had ‘‘heart surgery’’ when she was younger, but she was not sure what type, and that her grandmother could never participate in many of the family activities. The athlete had an ECG that was unremarkable except for a borderline prolonged QTc at 468ms. Upon further investigation into the family history, it was found that her grandmother had undergone myomectomy for hypertrophic cardiomyopathy. Genetic testing had revealed a causative variant in MYH7. The athlete had an echocardiogram which was unremarkable, and genetic testing that showed she was not an MYH7 variant carrier. The athlete continued to row but will have yearly ECG because of the borderline QT prolongation.

Family History of Hypertrophic or Dilated Cardiomyopathy from Family History Section Frederick Dewey, MD

Question 8a. Does anyone in your family have any of the following: hypertrophic cardiomyopathy (HCM) or dilated cardiomyopathy (DCM)? The purpose of this question is to probe specifically for the more common familial cardiomyopathies that can lead to sudden death on the playing field. Familial HCM exists with an estimated prevalence of 1/500 in the general population, and many affected patients are asymptomatic at the time of diagnosis (1,2). Familial DCM accounts for an estimated 20% to 50% of cases of ‘‘idiopathic’’ DCM. These conditions have highly variable penetrance (that is, the proportion of genetically at-risk patients with clinical disease), age of onset, and clinical presentations (3). Both diseases can be mimicked by acquired forms of heart disease such as hypertension and coronary artery disease in older athletes, and adaptive training-related changes may be difficult to distinguish from pathologic primary cardiomyopathy. Because these conditions are rare, many screening physicians are unfamiliar with the initial presentation and diagnostic workup, and the true diagnosis may be elusive or uncertain in some family members. For these reasons, it can be challenging to assess the true risk for familial HCM or DCM-related cardiovascular events. A series of follow-up questions can clarify family history and uncover characteristic clinical findings, suggesting a true genetic risk for HCM or DCM. Cardiovascular Preparticipation Evaluation


FOLLOW-UP QUESTIONS To determine the importance of a positive answer to these questions, the following questions should be asked:

1) Who in your family carries the diagnosis, and at what age was the diagnosis made? It is important to clarify how the affected family member is related to the patient. Familial HCM and DCM are passed on genetically through family members related by blood. HCM and DCM are generally inherited in an autosomaldominant manner. Thus, first-, second-, and third-degree relatives have approximately 50%, approximately 25%, and approximately 12.5% risk of inheriting the genetic risk for the disease, respectively. The presence of multiple clearly affected family members is strongly indicative of familial, rather than acquired HCM/DCM. Using generally descriptive terms (e.g., ‘‘abnormally enlarged or thickened heart’’) can help probe for family members with suspicious phenotypic manifestations of disease but no formal diagnosis. An earlier age of diagnosis (G40 years old) often suggests that the disease is symptomatic and inherited or that the disease was discovered via family screening for a clearly inherited condition. Late age of onset, particularly with concomitant chronic diseases such as hypertension or coronary artery disease, may suggest a nongenetic disease process.

2) How was the diagnosis made? A review of the data supporting a family member’s diagnosis is critical to interpreting a positive response. Diagnoses made at the time of autopsy, particularly with supporting genetic information, are generally correct. The presence of characteristic electrocardiography (ECG) findings (such as T-wave inversion in precordial leads) and abnormal echocardiographic structural and diastolic parameters, particularly with outflow tract obstruction or systolic anterior motion of the mitral valve, is highly indicative of HCM. Global hypokinesis on echocardiogram or MRI at an early age, or primarily right ventricular systolic dysfunction, is indicative of familial DCM. MRI evidence of myocardial fibrosis can indicate HCM or DCM.

3) Has anyone in your family had genetic testing for these heart conditions? If genetic testing has been performed, it is likely that the clinical suspicion for familial HCM or DCM is high. Family members affected by hypertensive, ischemic, or other acquired forms of cardiac hypertrophy or DCM will not have had such testing. The sensitivity of clinical genetic testing for causative mutations in HCM is approximately 10% to 60% and in DCM is G20%, even among patients with clearly familial forms of cardiomyopathy. Thus, genetic testing can strengthen a clinical diagnosis and risk-stratify other family members. Therefore, genetic testing cannot be used to rule out the disease, and a negative test result should not be interpreted as evidence that the disease is not genetic. In some cases, the disease-causing mutation in the family is known and the patient will have had genetic testing for that mutation. If the patient has tested negative for the mutation, it is reasonable to assume that the patient is at the same risk for disease as a patient without family history of HCM or DCM. Consultation with an experienced www.acsm-csmr.org

cardiovascular geneticist and/or genetic counselor is strongly recommended.

4) Have any of your family members had pacemakers or defibrillators implanted? Patients will often confuse pacemakers with defibrillators and vice versa. However, the presence of either in a family member who carries a known diagnosis or suspicion for HCM or DCM can be an important indication of a familial cardiomyopathy and associated risk for sudden death. In DCM, the presence of a pacemaker may suggest conduction system disease associated with inherited forms of disease. The presence of an ICD in a family member can indicate high risk for or previous sudden cardiac arrest in a family member with either disease and can be a clue to arrhythmic risk in the family.

5) Has anyone in your family died unexpectedly or had surgery for these conditions? The presence of family members with a history of surgical procedures done specifically for familial HCM, such as surgical myomectomy, percutaneous alcohol septal ablation, mitral valve repair, or heart transplant at an early age is highly indicative of a familial form of disease. Similarly, a family history of sudden death, particularly at an early age, is more indicative of familial than acquired forms of HCM/DCM. WHEN TO REFER All patients who are considering participating in competitive athletics and report a family history of HCM or DCM should be referred for evaluation by a cardiologist familiar with the clinical presentation, diagnostic workup, genetics, and screening recommendations for these disease conditions. ECG and echocardiography can miss up to half of the early presentations. An initially negative screening evaluation does not exclude risk for future development of disease, particularly among adolescents. Therefore, some experts recommend that repeat evaluation be performed yearly in adolescence and every 2 to 5 years in adulthood, depending on level of physical activity, for those who have documented HCM or DCM in the family. Genetic testing can, in some cases, reveal the genetic cause of disease. In families with known disease-causing mutations, such testing can help determine the true genetic risk. Such evaluation requires highly specialized knowledge of a very rapidly changing field and should be performed in consultation with an experienced cardiovascular geneticist and/or genetic counselor. KEY POINTS 1) HCM and DCM are relatively common inherited forms of cardiomyopathy with variable penetrance that are associated with increased risk of athletic sudden death. 2) HCM and DCM can be mimicked by acquired forms of heart disease, and it is critical to assess whether a positive response reflects a true genetic risk. 3) Review of primary data supporting the diagnosis in a family member and the pattern of inheritance can clarify genetic risk. Current Sports Medicine Reports


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4) All patients with first-, second-, or third-degree relatives affected by HCM or DCM should be evaluated with an ECG and echocardiogram and referred to a cardiologist familiar with diagnosis, genetics, and treatment of these conditions if abnormalities are found. CASE REPORTS Case Report 1 An 18-year-old athlete presented for a PPE prior to participating in competitive swimming. He is healthy, has no trouble keeping up with his peers in training, and has never had exertional chest discomfort, shortness of breath out of proportion to the level of activity, syncope, or presyncope with exercise. His physical examination results were unremarkable. He reported that his brother was diagnosed with HCM at age 16 by genetic testing, which revealed a wellestablished mutation in the gene MYH7 (p.R403Q). A family history reveals that his mother was diagnosed with HCM at age 44 based on family screening. There is no reported family history of sudden death. He had a normal echocardiogram and ECG result and had targeted testing for the MYH7 mutation and was found to not carry this genetic variant. He was allowed to compete in swimming. Case Report 2 A 20-year-old athlete reported for a PPE prior to the competitive basketball season. He is healthy, has not had symptoms of exertional chest pain, dyspnea, or syncope, and competes at a high level. He reported a family history of DCM diagnosed in his father at age 65 following two myocardial infarctions, the first at age 52 and necessitated coronary artery bypass surgery. Family history inquiry reveals that no other family members carry the diagnosis or have been told that they have abnormally enlarged or thickened hearts. There is no family history of sudden death. The DCM in his father was attributed to coronary artery disease, and the athlete was allowed to participate. References 1. Fokstuen S, Lyle R, Munoz A, et al. A DNA resequencing array for pathogenic mutation detection in hypertrophic cardiomyopathy. Hum. Mutat. 2008; 29:879Y85. 2. Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2011; 124:2761Y96. 3. Morita H, Rehm HL, Menesses A, et al. Shared genetic causes of cardiac hypertrophy in children and adults. N. Engl. J. Med. 2008; 358:1899Y908.

Family History of Inherited Arrhythmic Disease from Family History Section Marco Perez, MD

Question 8c. Does anyone in your family have an inherited arrhythmic condition such as long QT, Brugada, catecholaminergic polymorphic ventricular tachycardia (CPVT), short QT, or early repolarization/J wave syndrome? 340


This question is looking for familial disorders associated with exertional SCD that are primarily due to abnormalities in ion channels and are not typically related to structural cardiac disease. Long QT syndrome is the most common of these disorders, with a prevalence of approximately 1 in 2,000 in the United States (4). Because these conditions are rare in the general population, the diagnosis is challenging, as screening physicians are unfamiliar with the presentation and diagnostic workup, patients are often asymptomatic, and characteristic findings on the electrocardiography (ECG) may be transient (3). FOLLOW-UP QUESTIONS To determine the importance of a positive answer to these questions, the following questions should be asked:

1) Who in your family carries the diagnosis, and at what age was the diagnosis made? These inherited arrhythmias usually follow an autosomaldominant pattern of inheritance, so an affected first-degree relative, such as a parent or sibling, confers a 50% risk of carrying the genetic susceptibility and a second-degree relative, such as a grandparent or aunt/uncle, a 25% risk. The yield of screening relatives that are further removed is lower. Although these conditions can present at any age, an early presentation increases the likelihood that the family member was indeed affected by an inherited condition.

2) How was the diagnosis made? The inherited arrhythmias are often misdiagnosed (5), which makes review of the family member diagnosis records essential. Long QT syndrome, for example, can be misdiagnosed due to an inaccurate measurement of the QT interval on the ECG or acquired causes such as the use of QT-prolonging medications can be attributed to genetics. Brugada syndrome, most common in people of Asian descent, can be misdiagnosed when the ECG is suspicious but does not meet the criteria for a classic ‘‘type I’’ Brugada pattern (1) or if the finding is made incidentally in an asymptomatic person. The diagnostic criteria for early repolarization/J wave syndrome has been particularly challenging due to disagreement over the exact definition of the ECG findings and because some of these findings are commonly found in the general population (2).

3) Has anyone in your family had genetic testing for these heart conditions? This question is most helpful when the diagnosis is clear clinically and a causative variant of high confidence has been identified. In this scenario, family members can be screened with targeted genetic testing. However, the yield of genetic testing for each of these conditions varies widely, with a causative variant identified in 75% of families with long QT syndrome and only 30% of families with Brugada syndrome (6). Interpretation of genetic testing results also can be complex due to insufficient information about each possible genetic variant. Consultation with an experienced inherited cardiac arrhythmia specialist and genetic counselor is strongly recommended if the athlete responds positively to this question. Cardiovascular Preparticipation Evaluation


4) All patients with first-, second-, or third-degree relatives affected by HCM or DCM should be evaluated with an ECG and echocardiogram and referred to a cardiologist familiar with diagnosis, genetics, and treatment of these conditions if abnormalities are found. CASE REPORTS Case Report 1 An 18-year-old athlete presented for a PPE prior to participating in competitive swimming. He is healthy, has no trouble keeping up with his peers in training, and has never had exertional chest discomfort, shortness of breath out of proportion to the level of activity, syncope, or presyncope with exercise. His physical examination results were unremarkable. He reported that his brother was diagnosed with HCM at age 16 by genetic testing, which revealed a wellestablished mutation in the gene MYH7 (p.R403Q). A family history reveals that his mother was diagnosed with HCM at age 44 based on family screening. There is no reported family history of sudden death. He had a normal echocardiogram and ECG result and had targeted testing for the MYH7 mutation and was found to not carry this genetic variant. He was allowed to compete in swimming. Case Report 2 A 20-year-old athlete reported for a PPE prior to the competitive basketball season. He is healthy, has not had symptoms of exertional chest pain, dyspnea, or syncope, and competes at a high level. He reported a family history of DCM diagnosed in his father at age 65 following two myocardial infarctions, the first at age 52 and necessitated coronary artery bypass surgery. Family history inquiry reveals that no other family members carry the diagnosis or have been told that they have abnormally enlarged or thickened hearts. There is no family history of sudden death. The DCM in his father was attributed to coronary artery disease, and the athlete was allowed to participate. References 1. Fokstuen S, Lyle R, Munoz A, et al. A DNA resequencing array for pathogenic mutation detection in hypertrophic cardiomyopathy. Hum. Mutat. 2008; 29:879Y85. 2. Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2011; 124:2761Y96. 3. Morita H, Rehm HL, Menesses A, et al. Shared genetic causes of cardiac hypertrophy in children and adults. N. Engl. J. Med. 2008; 358:1899Y908.

Family History of Inherited Arrhythmic Disease from Family History Section Marco Perez, MD

Question 8c. Does anyone in your family have an inherited arrhythmic condition such as long QT, Brugada, catecholaminergic polymorphic ventricular tachycardia (CPVT), short QT, or early repolarization/J wave syndrome? 340


This question is looking for familial disorders associated with exertional SCD that are primarily due to abnormalities in ion channels and are not typically related to structural cardiac disease. Long QT syndrome is the most common of these disorders, with a prevalence of approximately 1 in 2,000 in the United States (4). Because these conditions are rare in the general population, the diagnosis is challenging, as screening physicians are unfamiliar with the presentation and diagnostic workup, patients are often asymptomatic, and characteristic findings on the electrocardiography (ECG) may be transient (3). FOLLOW-UP QUESTIONS To determine the importance of a positive answer to these questions, the following questions should be asked:

1) Who in your family carries the diagnosis, and at what age was the diagnosis made? These inherited arrhythmias usually follow an autosomaldominant pattern of inheritance, so an affected first-degree relative, such as a parent or sibling, confers a 50% risk of carrying the genetic susceptibility and a second-degree relative, such as a grandparent or aunt/uncle, a 25% risk. The yield of screening relatives that are further removed is lower. Although these conditions can present at any age, an early presentation increases the likelihood that the family member was indeed affected by an inherited condition.

2) How was the diagnosis made? The inherited arrhythmias are often misdiagnosed (5), which makes review of the family member diagnosis records essential. Long QT syndrome, for example, can be misdiagnosed due to an inaccurate measurement of the QT interval on the ECG or acquired causes such as the use of QT-prolonging medications can be attributed to genetics. Brugada syndrome, most common in people of Asian descent, can be misdiagnosed when the ECG is suspicious but does not meet the criteria for a classic ‘‘type I’’ Brugada pattern (1) or if the finding is made incidentally in an asymptomatic person. The diagnostic criteria for early repolarization/J wave syndrome has been particularly challenging due to disagreement over the exact definition of the ECG findings and because some of these findings are commonly found in the general population (2).

3) Has anyone in your family had genetic testing for these heart conditions? This question is most helpful when the diagnosis is clear clinically and a causative variant of high confidence has been identified. In this scenario, family members can be screened with targeted genetic testing. However, the yield of genetic testing for each of these conditions varies widely, with a causative variant identified in 75% of families with long QT syndrome and only 30% of families with Brugada syndrome (6). Interpretation of genetic testing results also can be complex due to insufficient information about each possible genetic variant. Consultation with an experienced inherited cardiac arrhythmia specialist and genetic counselor is strongly recommended if the athlete responds positively to this question. Cardiovascular Preparticipation Evaluation


4) Has anyone in your family received treatment for this condition, such as medications or a pacemaker/ defibrillator? Conditions such as long QT syndrome and CPVT are usually treated with medications such as beta-blockers. Patients with any of the inherited arrhythmias who have high-risk features may have received a defibrillator, which is often confused for a ‘‘pacemaker’’ by family members. A positive response with a treatment consistent with the disease in question increases the likelihood of an accurate family history. WHEN TO REFER Patients who are considering participating in competitive athletics and report a family history of inherited arrhythmic diseases in a first- or second-degree relative should be referred for evaluation by a cardiologist and genetic counselor familiar with the clinical presentation, diagnostic workup, genetics, and screening recommendations of these disease conditions. Athletes are usually evaluated with modified ECG, exercise tests, and possibly chemical challenges when the family history is positive. Since initial testing can result in false negative testing result, athletes often require ongoing evaluation based on the suspected arrhythmia disorder, age, and exercise intensity. Centers specializing in family-based care of patients with inherited cardiomyopathy are well situated to provide longitudinal follow-up and screening of other family members. Finally, genetic testing may play a role in screening families where a causative variant has been identified. The interpretation of this testing requires highly specialized knowledge of a very rapidly changing field and should be performed in consultation with an experienced cardiovascular geneticist and/or genetic counselor. CASE REPORTS Case Report 1 A 19-year-old male college basketball player presents for a PPE. He denies ever having palpitations, dizziness, lightheadedness, or syncope and has normal physical examination results. He recognized ‘‘long QT syndrome’’ and stated that he thought his mother was diagnosed with this in the hospital a few months back. The athlete was referred to a cardiologist who performed a lying and standing ECG where his corrected QT interval was within normal limits. The mother’s hospital records were obtained, and she had been treated with azithromycin for pneumonia. An ECG revealed a QTc of 490 ms, and she was diagnosed with acquired long QT. After being taken off the antibiotics, her ECG normalized. Although there may be some degree of underlying genetic susceptibility to acquired long QT syndrome, family members are currently not required to undergo screening. The athlete was allowed to play basketball and will not require routine ECG; he should have an ECG checked when and after taking QT-prolonging medications. Case Report 2 A 14-year-old Asian candidate for the girl’s badminton team presented for a PPE at the community center. She was very healthy and had no history of palpitations or syncope. www.acsm-csmr.org

Her physical examination result was normal. When asked about family history, she immediately recognized the Brugada syndrome. Her paternal uncle had been diagnosed with Brugada syndrome and had a defibrillator placed, but her family did not think that she was at risk because her father’s ECG was normal. She was referred to a specialized inherited arrhythmia clinic where her modified Brugada ECG was found to be normal. The genetic counselor tracked down genetic test results from her uncle that showed a variant in SCN5A with a high degree of confidence for pathogenicity. The athlete and her father tested positive for this pathogenic variant. The athlete and father were advised to avoid sodium channel-blocking medications, to control fevers with acetaminophen, and to avoid excessive heat. With a normal ECG and no history of syncope, she was considered at relatively low, but higher than the average athlete, risk of sudden death during athletic activity. She chose to continue playing badminton. An automatic external defibrillator (AED) was arranged for all her practices and games. Her family also opted to take basic life support classes and purchase an AED for the home. She will have yearly ECG and report any syncope immediately. References 1. Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005; 111:659Y70. 2. Froelicher V, Wagner G. Introduction to Symposium on J wave patterns and a syndrome. J Electrocardiography. 2013; 46:1Y3. 3. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993; 88:782Y4. 4. Schwartz PJ, Stramba-Badiale M, Crotti L, et al. Prevalence of the congenital long-QT syndrome. Circulation. 2009; 120:1761Y7. 5. Taggart NW, Haglund CM, Tester DJ, Ackerman MJ. Diagnostic miscues in congenital long-QT syndrome. Circulation. 2007; 115:2613Y20. 6. Tester DJ, Will ML, Haglund CM, Ackerman MJ. Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing. Heart Rhythm. 2005; 2:507Y17.

Marfan History in Athletes from Family History Section David Liang, MD, PhD

Question 9. Does anyone in your family have Marfan syndrome? Marfan syndrome is a heritable disease due to mutations in the fibrillin-1 gene that leads to aortic root aneurysms and mitral valve prolapse. Those afflicted are at risk for aortic dissection and/or rupture that may be precipitated by the increased hemodynamic stress of exercise. The overall population prevalence is thought to be 1 in 5,000 to 1 in 10,000. Inheritance is autosomal dominant with near 100% penetrance. Approximately 25% to 33% of cases will represent spontaneous mutations, so over half of those with Marfan syndrome can be identified with this simple question. The reliability of this question is limited by the accuracy of the Marfan syndrome diagnosis in the family member with the syndrome. It is worthwhile to obtain details upon which the diagnosis was made. If only musculoskeletal features were noted in the affected relative and there is no Current Sports Medicine Reports


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4) Has anyone in your family received treatment for this condition, such as medications or a pacemaker/ defibrillator? Conditions such as long QT syndrome and CPVT are usually treated with medications such as beta-blockers. Patients with any of the inherited arrhythmias who have high-risk features may have received a defibrillator, which is often confused for a ‘‘pacemaker’’ by family members. A positive response with a treatment consistent with the disease in question increases the likelihood of an accurate family history. WHEN TO REFER Patients who are considering participating in competitive athletics and report a family history of inherited arrhythmic diseases in a first- or second-degree relative should be referred for evaluation by a cardiologist and genetic counselor familiar with the clinical presentation, diagnostic workup, genetics, and screening recommendations of these disease conditions. Athletes are usually evaluated with modified ECG, exercise tests, and possibly chemical challenges when the family history is positive. Since initial testing can result in false negative testing result, athletes often require ongoing evaluation based on the suspected arrhythmia disorder, age, and exercise intensity. Centers specializing in family-based care of patients with inherited cardiomyopathy are well situated to provide longitudinal follow-up and screening of other family members. Finally, genetic testing may play a role in screening families where a causative variant has been identified. The interpretation of this testing requires highly specialized knowledge of a very rapidly changing field and should be performed in consultation with an experienced cardiovascular geneticist and/or genetic counselor. CASE REPORTS Case Report 1 A 19-year-old male college basketball player presents for a PPE. He denies ever having palpitations, dizziness, lightheadedness, or syncope and has normal physical examination results. He recognized ‘‘long QT syndrome’’ and stated that he thought his mother was diagnosed with this in the hospital a few months back. The athlete was referred to a cardiologist who performed a lying and standing ECG where his corrected QT interval was within normal limits. The mother’s hospital records were obtained, and she had been treated with azithromycin for pneumonia. An ECG revealed a QTc of 490 ms, and she was diagnosed with acquired long QT. After being taken off the antibiotics, her ECG normalized. Although there may be some degree of underlying genetic susceptibility to acquired long QT syndrome, family members are currently not required to undergo screening. The athlete was allowed to play basketball and will not require routine ECG; he should have an ECG checked when and after taking QT-prolonging medications. Case Report 2 A 14-year-old Asian candidate for the girl’s badminton team presented for a PPE at the community center. She was very healthy and had no history of palpitations or syncope. www.acsm-csmr.org

Her physical examination result was normal. When asked about family history, she immediately recognized the Brugada syndrome. Her paternal uncle had been diagnosed with Brugada syndrome and had a defibrillator placed, but her family did not think that she was at risk because her father’s ECG was normal. She was referred to a specialized inherited arrhythmia clinic where her modified Brugada ECG was found to be normal. The genetic counselor tracked down genetic test results from her uncle that showed a variant in SCN5A with a high degree of confidence for pathogenicity. The athlete and her father tested positive for this pathogenic variant. The athlete and father were advised to avoid sodium channel-blocking medications, to control fevers with acetaminophen, and to avoid excessive heat. With a normal ECG and no history of syncope, she was considered at relatively low, but higher than the average athlete, risk of sudden death during athletic activity. She chose to continue playing badminton. An automatic external defibrillator (AED) was arranged for all her practices and games. Her family also opted to take basic life support classes and purchase an AED for the home. She will have yearly ECG and report any syncope immediately. References 1. Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005; 111:659Y70. 2. Froelicher V, Wagner G. Introduction to Symposium on J wave patterns and a syndrome. J Electrocardiography. 2013; 46:1Y3. 3. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993; 88:782Y4. 4. Schwartz PJ, Stramba-Badiale M, Crotti L, et al. Prevalence of the congenital long-QT syndrome. Circulation. 2009; 120:1761Y7. 5. Taggart NW, Haglund CM, Tester DJ, Ackerman MJ. Diagnostic miscues in congenital long-QT syndrome. Circulation. 2007; 115:2613Y20. 6. Tester DJ, Will ML, Haglund CM, Ackerman MJ. Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing. Heart Rhythm. 2005; 2:507Y17.

Marfan History in Athletes from Family History Section David Liang, MD, PhD

Question 9. Does anyone in your family have Marfan syndrome? Marfan syndrome is a heritable disease due to mutations in the fibrillin-1 gene that leads to aortic root aneurysms and mitral valve prolapse. Those afflicted are at risk for aortic dissection and/or rupture that may be precipitated by the increased hemodynamic stress of exercise. The overall population prevalence is thought to be 1 in 5,000 to 1 in 10,000. Inheritance is autosomal dominant with near 100% penetrance. Approximately 25% to 33% of cases will represent spontaneous mutations, so over half of those with Marfan syndrome can be identified with this simple question. The reliability of this question is limited by the accuracy of the Marfan syndrome diagnosis in the family member with the syndrome. It is worthwhile to obtain details upon which the diagnosis was made. If only musculoskeletal features were noted in the affected relative and there is no Current Sports Medicine Reports


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history of aortic disease or lens dislocation, then the reliability of the diagnosis is suspect because there is considerable overlap in musculoskeletal phenotype between those with and without Marfan syndrome. If the diagnosis was determined by autopsy or by someone with extensive experience diagnosing Marfan syndrome, the accuracy will be improved. The relationship to the affected family member also should be considered in determining the significance of an affected family member. Since inheritance occurs in an autosomaldominant fashion with near 100% penetrance, the presence of definitively unaffected family members along the genetic path connecting the subject under evaluation and the affected family members eliminates the significance of an affirmative response to this question. As an example, if a subject reports that a paternal cousin has been found to have Marfan syndrome with an enlarged aorta and the corresponding paternal uncle or aunt has been excluded appropriately for the presence of Marfan with echocardiogram and appropriate physical examination, then the risk that the subject has Marfan syndrome is not significantly greater than the general population and further testing is not needed based upon family history alone. Some cases of Marfan syndrome can have very subtle clinical manifestations so further reassurance can be derived by exploring history of the paternal grandparents since they also would have to be implicated with Marfan syndrome, if the risk of the subject under evaluation is to be influenced by the affected cousin. If a parent has definitive Marfan syndrome, the pretest probability that the athlete will have Marfan syndrome is 50%; thus, the sensitivity of a physical examination to exclude Marfan syndrome is not high enough to rely upon it to exclude the diagnosis. In this situation, an echocardiogram should be done before participation unless genetic testing information is available to exclude the possibility of genetic transmission. CASE STUDY A college student being evaluated prior to joining the volleyball team relates to you that her maternal cousin has been diagnosed with Marfan syndrome. Upon examination, she is tall and lanky, but without other stigmata of Marfan syndrome. Her cousin had aortic root replacement surgery and is very nearsighted making the diagnosis of Marfan syndrome fairly certain. With further probing along the path of relation, the cousin’s mother is in good health, has undergone a careful evaluation for Marfan syndrome, and has been found not to have Marfan syndrome. Her mother is also in good health. With this information, you can safely disregard the report of Marfan syndrome.

Physical Examination David Liang, MD, PhD

Question 10. Physical examination finding of diminished femoral pulse A subjectively diminished pulse on physical examination can be due to body habitus; however, it also can be a manifestation of impaired blood flow to the femoral arteries by either an aortic coarctation or narrowing within the iliofemoral arteries. 342


ADDITIONAL HISTORY QUESTIONS Significantly impaired flow into the femoral arteries will result in claudication with exercise. Other relevant symptoms include night sweats, malaise, and weight loss due to the systemic inflammatory process in arteritis. A history of abrupt pain in the chest, back, abdomen or pelvis may signal an arterial dissection. PHYSICAL EXAMINATION FOLLOW-UP This finding should prompt an assessment of the blood pressure in both upper and lower extremities. Normal systolic blood pressure in the legs is 10% to 20% higher than that in the arm. If the leg pressure is more than 10 mm Hg lower than the arm pressure, the possibility of an obstruction should be explored. Asymmetry in arm pressures also may be present since the origins of the subclavian arteries also can be affected by the underlying condition. Systemic hypertension is generally present with clinically significant coarctation. Presence and absence of bruits also should be assessed since this may mark an area of obstruction. Typical points that would result in impairing flow to the femoral arteries would include in the distal aortic arch from aortic coarctation or in narrowing of the iliac arteries. Aortic coarctation will typically result in a systolic murmur heard in the left infraclavicular area or under the left scapula. Obstruction in the iliac arteries may result in periumbilical bruits or bruits in the groin. In a young patient, the presence of abdominal bruits should raise a concern for arteritis as well as arterial dissections. ADDITIONAL TESTING Presence of aortic coarctation can usually be confirmed or refuted by transthoracic echocardiography. In the presence of coarctation, even if the resting gradient is fairly low, an exercise stress test should be completed to evaluate for occult exercise-induced hypertension since collateral blood flow may preserve lower extremity pressure at rest. Anatomical definition of the obstruction should be performed once the diagnosis is confirmed. Visualization by CT or MR angiography can determine the location, extent, and the nature of the obstruction. In this young population, MR angiography is preferable to avoid radiation exposure.


Question 11. Does the athlete exhibit the physical findings of Marfan syndrome? The diagnosis of Marfan syndrome is currently defined by the revised Ghent criteria (1), which include the assessment of musculoskeletal features in the scoring scheme. The physical manifestations are a result of joint laxity and overgrowth of the long bones. The common axial skeletal features seen are scoliosis (920-) and pectus deformities. The limbs and fingers are disproportionately longer, giving the appearance of arachnodactyly (‘‘spider fingers’’). Presence of overgrowth of the bones of the hand may be confirmed by the ability to wrap the hand around the opposing Cardiovascular Preparticipation Evaluation


















integument and underlying connective tissue also can manifest itself in hernias. All these features also can be present in the general population; thus, the presence of a single one of these features, unless particularly striking, should not warrant a more extensive evaluation for Marfan syndrome unless other symptoms or historical features raise suspicion for the diagnosis. However, when multiple characteristics are present, it is essential to investigate further with an echocardiogram and dilated slit lamp examination of the eyes to exclude the presence of more specific findings such as aortic root enlargement or intraocular lens dislocation. Often, a referral or consultation with a geneticist or a cardiologist familiar with Marfan syndrome evaluation and management will be helpful.

Figure 1: Walker-Murdoch wrist sign. Note the thumb covering the entire nail of the fifth digit ("little finger").

wrist and cover the entire distal portion of the fifth (little finger) to the distal interphalangeal joint (Walker-Murdoch sign) (Fig. 1) or by the ability to extend the distal portion of the thumb beyond the end of a closed fist (Steinberg sign) (Fig. 2). The disproportionate growth of the limbs can be assessed by comparing arm span with height with an arm span to height ratio 91.05 being abnormal for Caucasians and by comparing the upper segment length (portion of height above the symphysis pubis) with the lower segment length (portion of height below the symphysis pubis). The loss of the plantar arch also is common in Marfan syndrome and in its greatest severity will result in inward motion of the medial malleolus of the ankle as the arch collapses and in severe calcaneal valgus deformity. The skin will often have a soft velvety character and striae in unusual locations like the shoulders, lower back, over the knees, and on the stomach. The weakness of the

Reference 1. Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J. Med. Genet. 2010; 47:476Y85.

EDITORS Irfan M. Asif, MD Vice Chair, Research and Academics Director, Sports Medicine Fellowship Associate Professor Department of Family Medicine Greenville Health System/University of South Carolina Greenville [email protected] William O. Roberts, MD, MS, FACSM Professor, Family Medicine 1414 Maryland Avenue E St. Paul, MN 55106 [email protected] Michael Fredericson, MD, FACSM Professor, Stanford University [email protected] Victor F. Froelicher MD, FACC, FAHA, FACSM Professor of Cardiovascular Medicine and Orthopedics/ Sports Medicine Director Sports Cardiology Clinic, Stanford University Center for Inherited Cardiovascular Disease 870 Quarry Road Falk Cardiovascular Research Building Stanford, CA 94305-5406 email: [email protected] INVITED PARTICIPANTS Francis G. O’Connor, COL, MC, USA, FACSM Professor and Chair, Military and Emergency Medicine Medical Director, Consortium for Health and Military Performance Uniformed Services University of the Health Sciences

Figure 2: Steinberg thumb sign. Note the entire nail of bent thumb extending beyond the ulnar border of the hand. www.acsm-csmr.org

Aaron L. Baggish, MD, FACC Cardiovascular Performance Program Massachusetts General Hospital Current Sports Medicine Reports


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Meagan M. Wasfy, MD Sports Medicine Fellow Cardiovascular Performance Program Massachusetts General Hospital Ricardo Stein, MD [email protected] Jeffrey S. Kutcher, MD Associate Professor Director, Michigan NeuroSport Department of Neurology University of Michigan Aswathnarayan R. Manandhi, MD (Raghu Manandhi) Cardiology Division, Hartford Hospital Paul D. Thompson, MD, FACC Chief of Cardiology, Hartford Hospital Abhimanyu (Manu) Uberoi, MD Cardiology Fellow, Cedars Sinai Medical Center Benjamin D. Levine, MD Director, Institute for Exercise and Environmental Medicine S. Finley Ewing Jr. Chair for Wellness at Texas Health Presbyterian Dallas Harry S. Moss Heart Chair for Cardiovascular Research Professor of Medicine and Cardiology Distinguished Professorship in Exercise Science University of Texas SouthwesternMedical Center at Dallas Chad Asplund, MD, MPH, FACSM Medical Director, Student Health Services and Sports Medicine Associate Professor, Family Medicine

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Georgia Regents University Team Physician, Georgia Regents University, Paine College Augusta Greenjackets

STANFORD FACULTY Jonathan Myers, PhD Palo Alto VA Medical Center David Liang, MD, PhD Director, Stanford Marfan’s Clinic Marco Perez, MD Staff Electrophysiologist Stanford CV Genomics Rick Dewey, MD Cardiology Fellow and Genomics Program Researcher Stanford Cardiology Michael J. Khadavi, MD PM&R Resident, Sports Medicine Program Stanford George K. Lui, MD, FACC Medical Director, The Adult Congenital Heart Program at Stanford Stanford Hospital & Clinics and Lucile Packard Children’s Hospital Clinical Assistant Professor of Medicine Division of Cardiovascular Medicine Stanford University School of Medicine Jon-Emile Stuart Kenny, MD Pulmonary Fellow Stephen Ruoss, MD Professor of Medicine, Pulmonary and Critical Care Stanford University Medical Center

