Hypertrophic cardiomyopathy

Most common cardiomyopathy and the most frequent cause of sudden cardiac death in young people.

Presentation varies from asymptomatic to symptoms of heart failure.

Physical examination may be normal at rest. Auscultation along the left sternal border when the patient is standing after a brief period of exercise may elicit a murmur.

Family history may be present. Echocardiography should be used to screen first-degree family members.

Has a benign prognosis in the majority of patients.

Medical therapy with beta-blockers, calcium-channel blockers, or disopyramide is used in symptomatic patients.

A subset of patients with increased risk for sudden death should undergo defibrillator implantation.

Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterised by left ventricular hypertrophy (LVH) without an identifiable cause. It is the most common genetic heart disease as well as the most frequent cause of sudden cardiac death in young people.[1]Maron BJ, Olivotto I, Maron MS. The dilemma of left ventricular outflow tract obstruction and sudden death in hypertrophic cardiomyopathy: do patients with gradients reallydeserve prophylactic defibrillators? Eur Heart J. 2006 Aug;27(16):1895-7. https://academic.oup.com/eurheartj/article/27/16/1895/2887194 http://www.ncbi.nlm.nih.gov/pubmed/16818455?tool=bestpractice.com Given its prevalence in younger patients, HCM is frequently confused with athlete’s heart. In older patients, HCM may be misdiagnosed as hypertensive heart disease. Many patients will have no symptoms at the time of diagnosis and will be diagnosed following routine examination or family screening of an affected family member.[2]Wigle ED, Rakowski H, Kimball BP, et al. Hypertrophic cardiomyopathy: clinical spectrum and treatment. Circulation. 1995 Oct 1;92(7):1680-92. http://circ.ahajournals.org/content/92/7/1680.full http://www.ncbi.nlm.nih.gov/pubmed/7671349?tool=bestpractice.com [3]Wynne J, Braunwald E. The cardiomyopathies and myocarditides. In: Braunwald E, Zipes DP, Libby P, eds. Heart disease: a textbook of cardiovascular medicine. 5th ed. Philadelphia, PA: WB Saunders; 1997.