Opinion statement
Sudden cardiac death is the most devastating manifestation of hypertrophic cardiomyopathy (HCM) and often occurs in young and previously asymptomatic patients. Therefore, risk stratification for sudden death has a major role in the management of HCM and has acquired even greater relevance since the implantable cardioverter-defibrillator (ICD) has proved to be highly effective in preventing sudden death in this disease. The ICD is definitely indicated for secondary prevention of sudden death in patients with HCM who have survived a cardiac arrest with documented ventricular fibrillation, or experienced one or more episodes of sustained ventricular tachycardia. However, uncertainties persist regarding the precise selection of patients for primary prophylactic ICD implantation. A number of risk markers are used to assess the magnitude of risk, including family history of premature sudden death; extreme left ventricular (LV) hypertrophy (> 30 mm) in young patients; nonsustained ventricular tachycardia on Holter electrocardiographic recording; unexplained (not neurally mediated) syncope, particularly in young patients; and blood pressure decrease or inadequate increase during upright exercise. Multiple risk factors convey a definite increase in risk. However, a single risk factor such as family history of multiple sudden deaths, massive LV hypertrophy in a young patient, or frequent and/or prolonged runs of nonsustained ventricular tachycardia on Holter, may also justify consideration of a prophylactic ICD.
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References and Recommended Reading
Maron BJ, McKenna WJ, Danielson GK, et al.: American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J Am Coll Cardiol 2003, 42:1687–1713.
Spirito P, Seidman CE, McKenna WJ, Maron BJ: The management of hypertrophic cardiomyopathy. N Engl J Med 1997, 336:775–785.
Maron BJ: Hypertrophic cardiomyopathy. A systematic review. JAMA 2002, 287:1308–1320.
Elliott P, McKenna WJ: Hypertrophic cardiomyopathy. Lancet 2004, 363:1881–1891.
Spirito P, Autore C: Management of hypertrophic cardiomyopathy. BMJ 2006, 332:1251–1255.
Moon JCC, McKenna WJ, McCrohon JA, et al.: Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol 2003, 41:1561–1567.
Carsten R, Norbert MW, Michael JH, et al.: Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation 2005, 112:855–861.
Seidman JG, Seidman C: The genetic basis for cardiomyopathy: from mutation identification to mechanistic paradigms. Cell 2001, 104:557–567.
Arad M, Maron BJ, Gorham JM, et al.: Glycogen storage diseases presenting as hypertrophic cardiomyopathy. N Engl J Med 2005, 352:362–372.
Maron BJ, Shen WK, Link MS, et al.: Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med 2000, 342:365–373.
Maron BJ, Spirito P, Shen WK, et al.: Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA 2007, 298:405–412.
Spirito P, Bellone P, Harris KM, et al.: Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med 2000, 342:1778–1785.
Elliott PM, Gimeno B Jr, Mahon NG, et al.: Relation between severity of left ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet 2001, 357:420–424.
Monserrat L, Elliott PM, Gimeno JR, et al.: Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J Am Coll Cardiol 2003, 42:873–879.
Sadoul N, Prasad K, Elliott PM, et al.: Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation 1997, 96:2987–2991.
Maron MS, Olivotto I, Betocchi S, et al.: Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 2003, 348:295–303.
Elliott PM, Poloniecki J, Dickie S, et al.: Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 2000, 36:2212–2218.
Maron BJ, Mark Estes NA, Maron MS, et al.: Primary prevention of sudden death as a novel treatment strategy in hypertrophic cardiomyopathy. Circulation 2003, 107:2872–2875.
Spirito P, Maron BJ, Bonow RO, et al.: Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol 1987, 60:123–129.
Biagini E, Coccolo F, Ferlito M, et al.: Dilated-hypokinetic evolution of hypertrophic cardiomyopathy: prevalence, incidence, risk factors, and prognostic implications in pediatric and adult patients. J Am Coll Cardiol 2005, 46:1543–1550.
Harris KM, Spirito P, Maron MS, et al.: Prevalence, clinical profile and significance of the end-stage phase in a large hypertrophic cardiomyopathy cohort. Circulation 2006, 114:216–225.
Boriani G, Maron BJ, Shen WK, Spirito P: Prevention of sudden death in hypertrophic cardiomyopathy. But which defibrillator for which patient? Circulation 2004, 110:e438–e442.
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Autore, C., Quarta, G. & Spirito, P. Risk stratification and prevention of sudden death in hypertrophic cardiomyopathy. Curr Treat Options Cardio Med 9, 431–435 (2007). https://doi.org/10.1007/s11936-007-0037-y
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DOI: https://doi.org/10.1007/s11936-007-0037-y