A case of rheumatic mitral stenosis with subaortic ventricular septal defect and anomalous right coronary artery from left sinus Mohammed Idhrees Abdulsamath, Vivek Pillai, Bineesh Radhakrishnan, Varghese Paniker, Praveen Varma & Jayakumar Karunakaran Indian Journal of Thoracic and Cardiovascular Surgery ISSN 0970-9134 Volume 31 Number 2 Indian J Thorac Cardiovasc Surg (2015) 31:184-186 DOI 10.1007/s12055-014-0339-0
1 23
Your article is protected by copyright and all rights are held exclusively by Indian Association of Cardiovascular-Thoracic Surgeons. This e-offprint is for personal use only and shall not be self-archived in electronic repositories. If you wish to self-archive your article, please use the accepted manuscript version for posting on your own website. You may further deposit the accepted manuscript version in any repository, provided it is only made publicly available 12 months after official publication or later and provided acknowledgement is given to the original source of publication and a link is inserted to the published article on Springer's website. The link must be accompanied by the following text: "The final publication is available at link.springer.com”.
1 23
Author's personal copy Indian J Thorac Cardiovasc Surg (April–June 2015) 31(2):184–186 DOI 10.1007/s12055-014-0339-0
CASE REPORT
A case of rheumatic mitral stenosis with subaortic ventricular septal defect and anomalous right coronary artery from left sinus Mohammed Idhrees Abdulsamath & Vivek Pillai & Bineesh Radhakrishnan & Varghese Paniker & Praveen Varma & Jayakumar Karunakaran
Received: 9 April 2014 / Revised: 5 June 2014 / Accepted: 3 November 2014 / Published online: 21 November 2014 # Indian Association of Cardiovascular-Thoracic Surgeons 2014
Abstract Rheumatic mitral stenosis (MS) is common in India. Ventricular septal defect (VSD) is the commonest congenital heart disease seen clinically. The incidence of right coronary artery (RCA) arising from the left aortic sinus is 0.17 %. The presence of all the three lesions in a patient is likely to be extremely rare. We present a patient who had all the three lesions, and to the best of our knowledge, this is the first such case to be reported.
Keywords Ventricular septal defect . Right coronary artery . Rheumatic mitral stenosis
Introduction Rheumatic heart disease (RHD) is a common problem in India with an incidence of 0.2 to 1.1/1000. Nearly 43 to 50 % develop significant mitral stenosis (MS) without a documented history of rheumatic fever possibly because of subclinical carditis [1]. Ventricular septal defect (VSD) accounts for nearly 35 % of congenital heart disease among neonates in India [2]. Excluding bicuspid aortic valve, VSDs are the most common CHD in adults with an estimated prevalence of 0.3 per 1000 [5]. The presence of anomalous right coronary artery from opposite sinus (ACAOS) makes it the first case in literature with the above three clinical entities.
M. I. Abdulsamath (*) : V. Pillai : B. Radhakrishnan : V. Paniker : P. Varma : J. Karunakaran Department of Cardiovascular and Thoracic Surgery, Shree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India e-mail:
[email protected]
Case report A 50-year-old male who is a laborer by occupation presented with history of dyspnea (NYHA grade IV) and orthopnea of progressive nature since 5 years old. There was no past history suggestive of rheumatic fever or myocardial ischemia. He had no signs of congestive heart failure. On auscultation, a middiastolic murmur (2/6) and a pansystolic murmur (3/6) were heard. ECG showed atrial fibrillation and left axis deviation, and chest X-ray revealed an enlarged left atrium and signs of pulmonary venous hypertension. Antistreptolysin O (ASO) titer was negative. Echocardiogram revealed severe MS with mitral valve area of 0.9 cm2 with normally placed papillary muscles and thickened chordae. There was no supravalvar pathology. The left atrium was grossly enlarged (63 mm), and a subaortic VSD of 5 mm was present (Fig. 1). Moderate pulmonary arterial hypertension was present. Coronary angiogram showed anomalous origin of right coronary artery from left sinus (Fig. 2). Surgery was performed under moderate hypothermia with aorto-bicaval cannulation. The right coronary artery (RCA) arising from the left coronary sinus was seen coursing anterior to the pulmonary artery. The left atrium was opened along the Sondergaard’s groove. The mitral valve leaflets appeared yellowish and thickened causing severe stenosis with no supravalvular pathology. Both the papillary muscles appeared to be positioned normally. The chordae were thickened. The mitral valve was excised with preservation of the posterior leaflet, and mitral vavle replacement was done using a 31-mm bileaflet mechanical valve prosthesis. The VSD was approached through the right atrium and was closed by direct pledget supported polypropylene sutures on the right ventricular side of the septum. No intervention was done for his right ACAOS. The postoperative period was uneventful. Histopathology of the mitral valve was consistent with rheumatic disease. Pre-discharge echocardiogram revealed normal prosthetic valve function and no
Author's personal copy Indian J Thorac Cardiovasc Surg (April–June 2015) 31(2):184–186
Fig. 1 Transesophageal echocardiogram showing mitral stenosis (a) and subaortic ventricular septal defect (b)
residual VSD. At 6-month follow-up, the patient was asymptomatic and had a normal electrocardiogram.
Discussion Rheumatic valve disease results in permanent damage of the heart structures following rheumatic fever (RF). On autopsy examination of patients dying of acute RF, it was noted that all
185
the four valves had microscopic disease. But clinically, mitral valve involvement occurs in more than 90 % of the patients [1]. In developing countries, mitral stenosis (MS) progresses much more rapidly giving rise to a different clinical entity— juvenile mitral stenosis probably because of severe or/and repeated streptococcal infections, genetic influence, or economic conditions [4]. In nearly 50 % of individuals develop significant MS without prior history of RF, possibly because of subclinical carditis [1]. Congenital mitral stenosis has a wide spectrum affecting the chordae, papillary muscle (single or closely placed), supramitral ring, double orifice. Systematic echocardiographic assessment of the mitral valve needs to be done to identify the pathology including the supravalvular region, annulus, leaflet, commissures, chordae, and the papillary muscle. Most of the children present at early age, nearly 50 % die by the end of 6 months. If the stenosis is severe, they present shortly after birth requiring immediate intervention [7]. Mild to moderate disease can be managed medically while severe disease needs surgical management. Coronary artery anomalies have a prevalence of 1.4 %. The incidence of right coronary artery from left sinus is 0.17 % [6]. There are four different types based on the anatomical course—retroaortic, inter-arterial, prepulmonic, and subpulmonic. Symptoms of ischemia depend on the presence of an inter-arterial course, slit-like orifice, acute angle takeoff, and presence and length of intramural segment [8]. ACOAS of RCA is a benign condition. Treatment for the same remains controversial and includes passive observation, percutaneous intervention or surgery after evaluating the risk-benefit ratio. Patient anxiety needs to be allayed and, in itself, is not an indication for surgery. Association of VSD with rheumatic mitral stenosis in older patients is not often encountered. Only very few case reports exists [3]. A nonrestrictive VSD can lead to pulmonary arterial hypertension (PAH). If the patient develops MS early in life, the progression of PAH is hastened. In our patient, the VSD was restrictive, and with a slowly progressive MS, he presented to the hospital only at 50 years of age. Since there was an associated left to right shunt, he had a normal left ventricular dimension despite severe mitral stenosis. Since his preoperative ECG showed no signs of ischemia and considering the fact that it was a prepulmonic type of right ACAOS, we decided not to correct his coronary anomaly.
Conclusion
Fig. 2 Coronary angiogram showing the origin of right coronary artery from left aortic sinus
The existence of VSD and rheumatic mitral stenosis with an anomalous coronary origin is a rare entity. If the VSD is restrictive, it may not add to pulmonary hypertension until the mitral valve is severely stenotic. ACAOS is generally a
Author's personal copy 186
benign lesion which can be medically followed up except for ones with inter-arterial course which require intervention.
References 1. Krishna Kumar R, Tandon R. Rheumatic fever and rheumatic heart disease: the last 50 years. Indian J Med Res. 2013;137:643–58. 2. Khalil A, Aggarwal R, Thirupuram S, Arora R. Incidence of congenital heart disease among hospital live births in India. Indian Pediatr. 1994;31:519–27.
Indian J Thorac Cardiovasc Surg (April–June 2015) 31(2):184–186 3. Chiu C, Shyu K. Coexistent supracristal ventricular septal defect and severe rheumatic mitral stenosis in a middle-aged woman: a case report. FJJM. 2009;7:39–45. 4. Report of a WHO expert consultation: rheumatic fever and rheumatic heart disease, Geneva, 29 October–1November 2001 5. Minette MS, Sahn DJ. Ventricular septal defects. Circulation. 2006;114:2190–7. 6. Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. CatheterCardiovascDiagn. 1990;21:28–40. 7. Remenyi B, Gentles TL. Congenital mitral valve lesions: correlation between morphology and imaging. Ann Pediatr Card. 2012;5:3–12. 8. Gersony WM. Management of anomalous coronary artery from the contralateral coronary sinus. J Am Coll Cardiol. 2007;50:2083–4.
