[Apical hypertrophic cardiomyopathy: an element in the continuum of hypertrophic cardiomyopathies]. / La cardiomyopathie hypertrophique apicale: un élément du continuum des cardiomyopathies hypertrophiques.

ABSTRACT

Fourteen cases of apical hypertrophic cardiomyopathy (i.e. hypertrophy localized to the distal half of the left ventricular wall) are reported. There were 12 men and 2 women aged from 21 to 84 years. Only one of the patients presented, at first consultation, with severe functional symptoms, namely stage IV dyspnoea. ECG tracings were always abnormal, but the classical giant T waves were found in only 7 patients. In the 9 patients who had cardiac catheterization the left ventricular end-diastolic pressure was raised, and angiography showed an "ace of spades" diastolic image of the left ventricle with systolic obliteration of its tip. The distribution of parietal hypertrophy was best studied by two-dimensional echocardiography the left ventricular apex was affected alone in 7 patients and concomitantly with the adjacent segments of the left or right ventricle in the other 7 patients. A family study showed that 3 patients had a descendant with obstructive cardiomyopathy. At a 4.6 years' follow-up the course of the disease was usually favourable. Apical hypertrophic cardiomyopathy is not a particular entity but one of different possible forms of hypertrophic cardiomyopathy. It seems to be benign in most cases.