Clinical Review

Management of hypertrophic cardiomyopathy

BMJ 2006; 332 doi: http://dx.doi.org/10.1136/bmj.332.7552.1251 (Published 25 May 2006) Cite this as: BMJ 2006;332:1251
  1. Paolo Spirito, director (paolo.spirito@galliera.it)1,
  2. Camillo Autore, professor of cardiovascular medicine2
  1. 1 Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Via Volta 8, Genoa 16128,Italy
  2. 2 Unità Operativa di Cardiologia, Ospedale Sant'Andrea, Università di Roma “La Sapienza”, Via Grottarossa, 1035-1039, Rome 00189, Italy
  1. Correspondence to: P Spirito
  • Accepted 21 April 2006

Introduction

Hypertrophic cardiomyopathy is the most common familial genetic disease of the heart (1/500 to 1/1000), as well as the most common cause of sudden cardiac death in young people and athletes. Because a timely diagnosis may help to prevent sudden death, it is important for internists and general practitioners to be aware of the clinical features of the disease. The morphological and functional features of hypertrophic cardiomyopathy include marked and asymmetric left ventricular hypertrophy, a non-dilated left ventricular cavity, and preserved systolic function. Left ventricular outflow obstruction at rest is present in about 20% of patients.13 The clinical course is heterogeneous. Many patients remain asymptomatic throughout life, others develop severe heart failure or atrial fibrillation, and some die suddenly, often at a young age and in the absence of previous symptoms.13 We critically re-examine and place in perspective the most appropriate therapeutic strategies for the management of hypertrophic cardiomyopathy.

Sources and search criteria

This review is based on the main studies on hypertrophic cardiomyopathy published in recent years, as well as on the clinical experience of doctors and investigators with particular knowledge of this disease. The treatment strategies reported in the expert consensus document on hypertrophic cardiomyopathy from the American College of Cardiology and the European Society of Cardiology are also incorporated in this article.

Diagnosis

Hypertrophic cardiomyopathy is usually suspected after marked electrocardiographic abnormalities or a heart murmur have been identified during routine clinical evaluation, or because of recent development of dyspnoea or palpitations.13 The diagnosis is established by the echocardiographic identification of a hypertrophied and non-dilated left ventricle in the absence of other cardiovascular diseases capable of producing a similar magnitude of hypertrophy14 (fig 1). In a minority of patients with electrocardiographic abnormalities suggestive of hypertrophic cardiomyopathy, the echocardiogram may fail …

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