Prognosis in hypertrophic cardiomyopathy

One hundred thirty-six patients with hypertrophic cardiomyopathy were followed up for 1 to 17 years. Twenty-one patients had died, 14 of them suddenly, two from heart failure, two from cerebral embolism, and three from noncardiac causes. Life table analysis revealed that sudden death was significantly associated with young age less than 20 years (relative risk [rr] = 8.63, when compared with those greater than 40 years) and with positive Master's single two-step test (rr = 3.55). Heart failure was more frequent in patients with positive Master's single test (rr = 4.27) and with left ventricular end-diastolic pressure greater than 20 mm Hg (rr = 2.58). Atrial fibrillation, observed in 15 patients, was a poor prognostic sign, resulting in five cardiac deaths and seven heart failures. In contrast, prognosis was favorable in patients with apical hypertrophy with giant negative T wave. Thus Japanese patients with hypertrophic cardiomyopathy showed a prognosis consistent with Western patients, except for excellent outcome of apical hypertrophy.