Cardiomyopathy
Unusual Features of Apical Hypertrophic Cardiomyopathy

https://doi.org/10.1016/j.amjcard.2009.11.037Get rights and content

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.

Section snippets

Methods

Over a 5-year period from 2003 to 2008, 32 consecutive patients with apical HC were retrospectively identified in the cardiology department of a teaching hospital without a specialized HC clinic. The study was approved by the Human Ethics Committee of the Central Sydney Area Health Service (Concord Hospital Division). Presenting symptoms and associated clinical features were characterized, and cardiovascular morbidity and mortality were determined on follow-up. Electrocardiograms were examined

Results

The clinical characteristics of our patients are listed in Table 1. Our cohort was elderly, with a mean age of 71 years. The mean follow-up of the patients was 4 ± 3 years (range 1 to 26), and 2 patients were lost to follow-up. The patients were unrelated apart from 2 who were related as father and daughter. Twenty-two patients had hypertension at the time of presentation. There were 6 deaths in the cohort, including 2 after atrial fibrillation–related hemiplegic strokes and 2 from progressive

Discussion

To our knowledge, this is the first and largest case series characterizing apical HC and its morbidity in an elderly non-Japanese population. In a general hospital without a referral bias to a specialized HC clinic, apical HC appears to be a disease of the elderly that carries an adverse prognosis, especially in the presence of atrial fibrillation. The diagnosis, when not carefully considered, can be missed. Apical HC in the elderly has unique cardiovascular characteristics, such as late

References (22)

  • Y. Suganuma et al.

    Clinical characteristics and cardiac events in elderly patients with apical hypertrophic cardiomyopathy

    Nippon Ronen Igakkai Zasshi

    (1997)
  • Cited by (0)

    View full text