REVIEW
Europace (2018) 0, 1–11 doi:10.1093/europace/euy064
Atrial fibrillation in patients with inherited cardiomyopathies Cynthia Yeung, Andres Enriquez, Laiden Suarez-Fuster, and Adrian Baranchuk* Kingston General Hospital, Queen’s University, Kingston, ON K7L 2V7, Canada Received 8 January 2018; editorial decision 11 March 2018; accepted 13 March 2018
Atrial fibrillation (AF) often complicates the course of inherited cardiomyopathies and, in some cases, may be the presenting feature. Each inherited cardiomyopathy has its own peculiar pathogenetic characteristics that can contribute to the development and maintenance of AF. Atrial fibrillation may occur as a consequence of disease-specific defects, non-specific cardiac chamber changes secondary to the primary illness, or a combination thereof. The presence of AF can denote a turning point in the progression of the disease, promoting clinical deterioration and increasing morbidity and mortality. Furthermore, the management of AF can be particularly challenging in patients with inherited cardiomyopathies. In this article, we review the current information on the prevalence, pathophysiology, risk factors, and treatment of AF in three different inherited cardiomyopathies: hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia/ cardiomyopathy, familial dilated cardiomyopathy, and left ventricular non-compaction cardiomyopathy.
................................................................................................................................................................................................... Keywords
Atrial fibrillation • Inherited cardiomyopathies • Hypertrophic cardiomyopathy • Arrhythmogenic right ventricular dysplasia/cardiomyopathy • Familial dilated cardiomyopathy • Left ventricular non-compaction cardiomyopathy
Introduction Atrial fibrillation (AF) is the most common sustained arrhythmia in clinical practice, affecting 1–4% of the general population,1–3 and is responsible for considerable morbidity and mortality, primarily because of an increased risk of stroke and heart failure.4 Atrial fibrillation often complicates the course of inherited cardiomyopathies and, in some cases, may be the presenting feature.5 Inherited cardiomyopathies, due to mutations in genes encoding specific structural proteins, are associated with atrial remodelling, histological changes, and modifications in atrial action potential characteristics that may mediate an increased risk of AF.6 Each inherited cardiomyopathy has its own peculiar pathogenetic characteristics that can be the basis of AF. Atrial fibrillation may occur as a consequence of disease-specific defects and/or non-specific cardiac chamber changes secondary to the primary illness.7 The management of AF is usually different in patients with inherited cardiomyopathies.6 In this article, we review the current information on the prevalence, pathophysiology, risk factors, and treatment of AF in three different inherited cardiomyopathies: hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), familial dilated
cardiomyopathy (FDCM), and left ventricular non-compaction cardiomyopathy (LVNC) (Table 1).
Methods This is a non-systematic comprehensive review of AF in HCM, ARVD/C, FDCM, and LVNC. A thorough EMBASE database and PubMed search was performed in order to identify original manuscripts focusing on this topic and published between 1990 and 2017. Selected study papers, recently published review articles, editorials from peer-reviewed journals, and case reports constituted the literature reference types of this manuscript.
Hypertrophic cardiomyopathy Atrial fibrillation prevalence in hypertrophic cardiomyopathy Atrial fibrillation is the most common sustained arrhythmia in HCM, with a prevalence of 20–30% and an annual incidence of 2% per year, which is four to six times more frequent than the general
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Table 1
Summary table of atrial fibrillation in inherited cardiomyopathies
Condition
Prevalence
Pathophysiology
Risk factors
Treatment
Hypertrophic cardiomyopathy
20–30%
Diastolic dysfunction, myocardial ischaemia, autonomic dysregu-
MYH7 and ACE gene mutations, increased age, large left atrial
Anticoagulation, amiodarone and sotalol, catheter ablation,
....................................................................................................................................................................................................................
lation, atrial fibrosis Arrhythmogenic right
11–30%
Desmosomal dysfunction, atrial
size, impaired left atrial function Large right atrium/left atrium/
cardiac implantable electronic devices Anticoagulation, antiarrhythmic
ventricular dysplasia/
remodelling, atrial scarring/
right ventricle size, moderate-
drugs, b-blockers, catheter
cardiomyopathy
fibrofatty replacement
severe tricuspid regurgitation, right ventricular dysfunction,
ablation, implantable cardioverter-defibrillator
atrial flutter, hypertension, Familial dilated
33%
cardiomyopathy
Conduction dysfunction preceding mechanical dysfunction
increased age, male gender NKX2-5 and TTNtv mutations, multiple genetic variants in core dilated cardiomyopathy genes
LVNC
Adults: 1–29%;
Adults: underlying myopathy, left atrial dilation, ion channel changes;
Children: 2–13% Children: atrioventricular accessory pathway
Increased age, exertional dyspnoea, diabetes mellitus, and
Anticoagulation, rhythm/rate control, catheter ablation, implantable cardioverterdefibrillator Anticoagulation (with vitamin K antagonists), Class III antiar-
heart failure; ECG abnormal-
rhythmic drugs, b-blockers,
ities, valvular abnormalities, lateral wall LVNC, more
catheter ablation, implantable cardioverter-defibrillator
extensive LVNC, worse left ventricular fractional shortening ECG, electrocardiogram; LVNC, left ventricular non-compaction cardiomyopathy.
population.8,9 Asymptomatic AF often occurs in patients with HCM. Device interrogation in patients with HCM and implantable cardioverter-defibrillators (ICDs) demonstrated clinically silent AF in 24%. In all, 44% of patients with clinically silent AF progressed to symptomatic AF, with an average progression time of 4 years.10 Another study by the same group reported that 26% of patients with HCM and AF developed permanent AF, while 74% were confined to symptomatic paroxysmal AF.11 The occurrence of AF represents a turning point in the course of HCM, leading to clinical deterioration, impaired quality of life, and increased mortality and morbidity.8,12,13 Atrial fibrillation in the context of HCM may contribute to the genesis of syncope.14–17 Atrial fibrillation is associated with an increased risk of death when compared to patients in sinus rhythm, mainly driven by an excess stroke and heart failure-related mortality.18,19 In a meta-analysis by Guttmann et al.9 (including 7381 patients from 33 studies), the prevalence and incidence of thrombo-embolic events was 27% and 3.75% per year, respectively, which is comparable to a general AF population with a CHA2DS2-Vasc score >_2.
Pathophysiology of atrial fibrillation in hypertrophic cardiomyopathy Factors related to the development of AF in HCM include diastolic dysfunction, myocardial ischaemia, and autonomic dysregulation.8 Diastolic dysfunction is likely the most relevant factor, considering that it is a prominent feature of the disease and its association with incident AF has been previously demonstrated in large prospective
cohorts.20 Diastolic dysfunction may increase the risk of AF through different mechanisms, including increased atrial afterload, atrial myocyte stretching, and increased wall stress as a result of atrial dilation.21 Atrial fibrosis is also prominent in HCM,22 likely as consequence of left atrial (LA) overload and stretching, but is possible that a primary atrial myopathy directly related to the genetic defect may also be present in these patients. Conversely, an alternative pathophysiological mechanism of AF in HCM may be LA overload due to altered left ventricular (LV) relaxation, secondary to LV fibrosis.7 In a necropsy study of HCM patients, the degree of interstitial fibrosis was significantly greater in individuals with concomitant AF compared to those without AF.23 Atrial scarring may serve as a substrate for slow conduction and intra-atrial re-entry and may increase vulnerability to non-pulmonary vein triggers for AF.24
Risk factors for atrial fibrillation in hypertrophic cardiomyopathy Hypertrophic cardiomyopathy is a genetic disease of the myocardium, characterized by unexplained cardiac hypertrophy, myocyte disarray, and fibrosis, caused by mutations in genes encoding sarcomeric proteins.25,26 Mutations in the myosin heavy chain beta (MYH7) gene and the angiotensin-converting enzyme (ACE) gene have been associated with the development of AF in patients with HCM.27,28 Furthermore, several clinical variables have been found to be associated with AF occurrence in HCM: (i) clinical characteristics: age,
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NYHA functional Class (III–IV), hypertension, vascular disease, obstructive sleep apnoea; (ii) electrocardiographic parameters: P-wave duration, ST changes, premature ventricular contractions; (iii) echocardiographic parameters: LA diameter and volume index, LA fractional shortening, LA strain rate, posterior wall thickness, LV size, LV outflow tract (LVOT) gradient; (iv) others: late gadolinium enhancement on magnetic resonance imaging, LV fibrosis, and N-terminal pro-brain natriuretic peptide.8,9,13,19,29–35 The most consistent AF predictors across studies include an increasing age, greater LA dimensions, and impaired LA function.8,36,37 In a large cohort of 480 patients with HCM, Olivotto et al.13 documented that AF occurred in 107 patients (22%) over 9.1 ± 6.4 years. On multivariate analysis, the most powerful predictor was a LA dimension >_45 mm [odds ratio (OR) 3.6; 95% confidence interval (CI) 2.6–5.7; P < 0.0001], followed by age at diagnosis >50 years (OR 2.8; 95% CI 1.8–4.5; P < 0.001), and NYHA Class III or IV (OR 3.8; 95% CI 1.8–8.0; P < 0.005). Besides being a predictor for AF, LA size is an independent predictor for thrombo-embolic stroke in patients with HCM.12 In another study including 427 consecutive patients with HCM, Maron et al.38 identified LA ejection fraction (_118 mL), and age (>_40 years) to be independently associated with AF. Current guidelines recommend that patients in sinus rhythm with LA diameter >_45 mm should undergo 6–12 monthly 48-h ambulatory electrocardiogram monitoring to detect AF.39
Treatment of atrial fibrillation in the context of hypertrophic cardiomyopathy Atrial fibrillation can result in haemodynamic and clinical decompensation due to the loss of atrial contraction and fast ventricular rate. Therefore, maintenance of sinus rhythm is highly desirable, and an aggressive rhythm control strategy is recommended.40 Due to the significant LV hypertrophy and potential for proarrhythmia in patients with HCM, Class III antiarrhythmic drugs, such as amiodarone and sotalol, are the first-line options.11,41–43 Disopyramide [associated with beta blockers (BBs)] is used in patients with LVOT obstruction.39 In one study of 46 patients with HCM and AF, sinus rhythm was restored in 29 patients (63%), and maintained in 22 of those patients, with amiodarone over a 5.5 follow-up period.44 A case of a patient with HCM and AF reversed with amiodarone is presented in Figure 1. However, the adverse effects profile of amiodarone is of concern with respect to its use in young patients.40 No available data exist regarding other Class III antiarrhythmics such as dofetilide and dronedarone. If rhythm control is not feasible, rate control with BBs is advised (atenolol, nadolol, metoprolol, or bisoprolol in the presence of a preserved left ventricular ejection fraction (LVEF); bisoprolol or carvedilol in the presence of systolic dysfunction) and verapamil or diltiazem (only with preserved LVEF).43 However, although guidelines have recommended rate/rhythm control, a recent retrospective longitudinal cohort study by Guttmann et al.35 (of 4248 patients with HCM without pre-existing AF, of which 740 developed AF) found that antiarrhythmic therapy does not prevent AF in the long-term. The role of catheter in patients with drug-refractory AF has been reported from several different centres.45–47 Maintenance of sinus rhythm has been achieved in up to two-thirds of patients, although
repeat procedures or continuation of antiarrhythmic medications are often necessary.48–51 A systematic review of 241 HCM patients who underwent catheter ablation for AF reported that AF recurrence ranged from 0 to 66% (median 35–40%).50 Patients with HCM are approximately twice as likely to have a relapse after a single ablation than those without HCM.48 Atrial fibrillation ablation failure in patients with HCM was not associated with LA wall thickness.52 Notably, QTc prolongation is an independent predictor of arrhythmia recurrence in HCM patients undergoing AF ablation and might be useful for identifying those patients likely to have a better outcome following the procedure.53 The best candidates for ablation are young patients (_22.1 mm/m2, measured in the apical four-chamber view.62 Other echocardiographic predictors include LA dilation,59,62 RV enlargement >250 mL,61 moderate to severe tricuspid regurgitation,13 and RV dysfunction (defined as a RV fractional area change G, p.L189R lamin A/C mutation in identical twins with dilated cardiomyopathy. 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