Unusual features of apical hypertrophic cardiomyopathy

Apical hypertrophic cardiomyopathy (HC) is commonly regarded as a relatively benign condition of young to middle-aged Japanese men. Apical HC in a predominantly Caucasian population is not well characterized. The cardiovascular characteristics, morbidity, and mortality of a series of elderly, predominantly Caucasian subjects with apical HC are described. Thirty-two consecutive patients with apical HC (mean age 71 years, 15 men) were identified from a teaching hospital without a specialized HC clinic. Twenty-three subjects were Caucasian, 8 were Asian, and none Japanese. Twenty-two patients had coexistent hypertension. Six patients had documented late evolution of apical HC on electrocardiography and echocardiography up to 5 years after previous documented normal left ventricular morphology on echocardiography. The diagnosis of apical HC was initially missed in 7 patients because of inadequate image quality of the left ventricular apex and a lack of awareness of the condition. The correct diagnosis was assigned to all 7 patients after repeat echocardiography. Six of 13 patients who underwent coronary angiography had associated coronary artery fistulae. One patient required an implantable defibrillator for exertional syncope. Ten of the patients developed atrial fibrillation, 6 of whom had complicating thromboembolic events. Of the 6 deaths in the cohort, 2 followed atrial fibrillation-related hemiplegic strokes, and 2 followed progressive heart failure. In conclusion, apical HC in a teaching hospital without a specialized HC clinic and in a predominantly Caucasian population is a disease of the elderly. Documented late morphologic evolution is not uncommon, with a high incidence of coronary fistulae and morbid atrial fibrillation.