The patient is a 46-year-old female with marked myocardial hypertrophy and heart failure

Abstract

A 46-year-old female patient sought medical assistance because of dyspnea on minimum exertion.The patient first had dyspnea and edema during pregnancy at the age of 29 years, when heart disease was diagnosed. After the delivery, she remained asymptomatic and underwent cardiological assessment. An echocardiogram at that time (July '80) showed asymmetrical myocardial hypertrophy and alteration in the mobility of the anterior cusp of the mitral valve, suggesting a disorder in ventricular compliance. The measures are shown in table I. The patient was regularly followed up with clinical and echocardiographic evaluations (table I).After 2 years, the patient underwent cardiac catheterization (January '82), which revealed normal coronary arteries and right and left ventricular hypertrophy. Simultaneous biventricular injection did not show disproportion between the thickness of the interventricular septum and that of the posterolateral wall of the left ventricle (table II).The patient's brother had hypertrophic cardiomyopathy and underwent heart transplantation due to heart failure. The patient was informed that her heart disease was familial and she remained asymptomatic.One year ago she was diagnosed with diabetes mellitus, and dyspnea triggered by exertion appeared and progressively increased.On electrocardiogram (August '96), she had sinus rhythm, a heart rate of 103 bpm, supraventricular extrasystoles, SAP +90° forward, QRS axis -60° forward, right atrium overload, left anterosuperior division bundle-branch block, anterior position of the QRS loop, presence of deep Q-waves in the V5, V6, I and aVL leads, and decrease in the left ventricle potentials in these same leads ( fig. 1).On echocardiogram (August '96), she had concentric myocardial hypertrophy with no signs of obstruction of the left ventricle outflow tract. In spite of the preserved dimensions of the left ventricle (table I), akinesia of a small portion of the inferior wall of the left ventricle and marked hypokinesia of the posterolateral wall of the left ventricle existed.A new electrocardiogram (May '97) showed sinus rhythm, a heart rate of 100 bpm, QRS axis forward and undetermined in the frontal plane, biatrial overload, and a