Objective To investigate prognosis and prognostic factors in patients with hypertrophic cardiomyopathy (HCM) in Japan.
Design A nationwide epidemiological study.
Setting Hospitals selected randomly from among all hospitals in Japan.
Patients Clinical and epidemiological information for 2155 patients with HCM were collected in 1999. Main outcome measures: Patients were classified on the basis of baseline prognostic factors. Survival rates up to 5 years were calculated by Cox's proportional hazard model for 1605 patients.
Results During the follow-up period 241 deaths were recorded. The crude 5-year survival rate for the entire cohort was 86% (95% confidence interval [CI]: 84-88), and annual mortality ranged from 2.2% to 3.0%. A higher cardiothoracic ratio on chest X-ray (hazard ratio [HR] 1.61 [95%CI: 1.26-2.05] with 1 standard deviation [6.2%] increase), a lower left ventricular ejection fraction (HR 1.42 [95%CI: 1.20-1.69] with 1 standard deviation [13%] decrease), and the presence of left bundle branch block (HR 3.14; 95%CI: 1.28-7.71) were independently associated with a poorer prognosis, while the presence of apical hypertrophy at baseline (HR 0.58; 95%CI: 0.36-0.92) predicted a better chance of survival.
Conclusions The nationwide survey of patients with hypertrophic cardiomyopathy yielded important information on its prognosis and prognostic factors. These observations afford, for the first time, a measure of risk stratification in patients with HCM in Japan.
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