Apical hypertrophic cardiomyopathy and left ventricular non-compaction: two faces of the same disease

摘要

A 15-year-old male came to our clinic because his father had died suddenly with a previous diagnosis of apical hypertrophic cardiomyopathy at age 38. His father's records said he had congestive heart failure, a maximal left ventricular wall thickness of 28 mm, biventricular systolic dysfunction and left atrial hypertrophy on echocardiography. A female cousin of the father had had a heart transplant because of restrictive hypertrophic cardiomyopathy with severe heart failure (the explanted heart showed hypertrophy, myocyte disarray and fibrosis). At 18 years of age, our patient was asymptomatic on normal physical examination. The ECG demonstrated sinus rhythm with biventricular hypertrophy. The echocardiogram showed a normal wall thickness in the basal and mid-left ventricular segments and prominent trabeculations in the apical posterior, inferior and lateral walls.