Hypertrophic cardiomyopathy: Prevalence, pathophysiology, and management of concurrent atrial arrhythmias
- Martin S Maron, MD
Martin S Maron, MD
- Assistant Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- Samuel Lévy, MD
Samuel Lévy, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Cardiology
- University of Marseille, France
- William J McKenna, MD
William J McKenna, MD
- Section Editor — Myopericardial Disease
- Professor of Cardiology
- University College, London
Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease most often (60 to 70 percent) caused by mutations in one of several sarcomere genes that encode components of the contractile apparatus. (See "Hypertrophic cardiomyopathy: Gene mutations and clinical genetic testing".)
HCM is characterized by left ventricular hypertrophy (LVH) of various morphologies, with a wide array of clinical manifestations and hemodynamic abnormalities (figure 1). Depending in part upon the site and extent of cardiac hypertrophy, patients with HCM can develop one or more of the following abnormalities:
●Myocardial ischemiaTo continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Robinson K, Frenneaux MP, Stockins B, et al. Atrial fibrillation in hypertrophic cardiomyopathy: a longitudinal study. J Am Coll Cardiol 1990; 15:1279.
- Olivotto I, Cecchi F, Casey SA, et al. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation 2001; 104:2517.
- Rowin EJ, Orfanos A, Estes NA, et al. Occurrence and Natural History of Clinically Silent Episodes of Atrial Fibrillation in Hypertrophic Cardiomyopathy. Am J Cardiol 2017.
- Tani T, Tanabe K, Ono M, et al. Left atrial volume and the risk of paroxysmal atrial fibrillation in patients with hypertrophic cardiomyopathy. J Am Soc Echocardiogr 2004; 17:644.
- Ohtani K, Yutani C, Nagata S, et al. High prevalence of atrial fibrosis in patients with dilated cardiomyopathy. J Am Coll Cardiol 1995; 25:1162.
- Savage DD, Seides SF, Maron BJ, et al. Prevalence of arrhythmias during 24-hour electrocardiographic monitoring and exercise testing in patients with obstructive and nonobstructive hypertrophic cardiomyopathy. Circulation 1979; 59:866.
- Stafford WJ, Trohman RG, Bilsker M, et al. Cardiac arrest in an adolescent with atrial fibrillation and hypertrophic cardiomyopathy. J Am Coll Cardiol 1986; 7:701.
- Spirito P, Chiarella F, Carratino L, et al. Clinical course and prognosis of hypertrophic cardiomyopathy in an outpatient population. N Engl J Med 1989; 320:749.
- Higashikawa M, Nakamura Y, Yoshida M, Kinoshita M. Incidence of ischemic strokes in hypertrophic cardiomyopathy is markedly increased if complicated by atrial fibrillation. Jpn Circ J 1997; 61:673.
- Suzuki M, Hirayama T, Marumoto K, et al. Paroxysmal atrial fibrillation as a cause of potentially lethal ventricular arrhythmia with myocardial ischemia in hypertrophic cardiomyopathy--a case report. Angiology 1998; 49:653.
- Boriani G, Rapezzi C, Biffi M, et al. Atrial fibrillation precipitating sustained ventricular tachycardia in hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol 2002; 13:954.
- Authors/Task Force members, Elliott PM, Anastasakis A, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014; 35:2733.
- MacIntyre C, Lakdawala NK. Management of Atrial Fibrillation in Hypertrophic Cardiomyopathy. Circulation 2016; 133:1901.
- Veselka J, Anavekar NS, Charron P. Hypertrophic obstructive cardiomyopathy. Lancet 2017; 389:1253.
- January CT, Wann LS, Alpert JS, et al. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation 2014; 130:e199.
- Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation 2011; 124:e783.
- Kirchhof P, Benussi S, Kotecha D, et al. 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS. Eur Heart J 2016; 37:2893.
- Spirito P, Seidman CE, McKenna WJ, Maron BJ. The management of hypertrophic cardiomyopathy. N Engl J Med 1997; 336:775.
- Liu X, Ouyang F, Mavrakis H, et al. Complete pulmonary vein isolation guided by three-dimensional electroanatomical mapping for the treatment of paroxysmal atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy. Europace 2005; 7:421.
- Kilicaslan F, Verma A, Saad E, et al. Efficacy of catheter ablation of atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy. Heart Rhythm 2006; 3:275.
- Gaita F, Di Donna P, Olivotto I, et al. Usefulness and safety of transcatheter ablation of atrial fibrillation in patients with hypertrophic cardiomyopathy. Am J Cardiol 2007; 99:1575.
- Bassiouny M, Lindsay BD, Lever H, et al. Outcomes of nonpharmacologic treatment of atrial fibrillation in patients with hypertrophic cardiomyopathy. Heart Rhythm 2015; 12:1438.
- Santangeli P, Di Biase L, Themistoclakis S, et al. Catheter ablation of atrial fibrillation in hypertrophic cardiomyopathy: long-term outcomes and mechanisms of arrhythmia recurrence. Circ Arrhythm Electrophysiol 2013; 6:1089.
- Zhao DS, Shen Y, Zhang Q, et al. Outcomes of catheter ablation of atrial fibrillation in patients with hypertrophic cardiomyopathy: a systematic review and meta-analysis. Europace 2016; 18:508.
- Providencia R, Elliott P, Patel K, et al. Catheter ablation for atrial fibrillation in hypertrophic cardiomyopathy: a systematic review and meta-analysis. Heart 2016; 102:1533.
- Chen MS, McCarthy PM, Lever HM, et al. Effectiveness of atrial fibrillation surgery in patients with hypertrophic cardiomyopathy. Am J Cardiol 2004; 93:373.
- Matsui Y, Fukada Y, Imai T, et al. Combined cox maze procedure, septal myectomy, and mitral valve replacement for severe hypertrophic obstructive cardiomyopathy complicated by chronic atrial fibrillation. Ann Thorac Cardiovasc Surg 2003; 9:323.
- Darby AE, Dimarco JP. Management of atrial fibrillation in patients with structural heart disease. Circulation 2012; 125:945.
- McKenna WJ, England D, Doi YL, et al. Arrhythmia in hypertrophic cardiomyopathy. I: Influence on prognosis. Br Heart J 1981; 46:168.
- Cecchi F, Olivotto I, Montereggi A, et al. Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population. J Am Coll Cardiol 1995; 26:1529.
- Siontis KC, Geske JB, Ong K, et al. Atrial fibrillation in hypertrophic cardiomyopathy: prevalence, clinical correlations, and mortality in a large high-risk population. J Am Heart Assoc 2014; 3:e001002.
- PREDISPOSING FACTORS
- CLINICAL MANIFESTATIONS
- Atrial fibrillation
- - Initial management
- - Long-term management
- Rhythm control
- Rate control
- Atrial flutter and other supraventricular tachycardias
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS