Clinical characteristics of and long-term outcome in chinese patients with hypertrophic cardiomyopathy

BACKGROUND: Data on the phenotypical pattern and natural history of hypertrophic cardiomyopathy in Chinese patients are very limited. The purpose of this study was to describe the clinical characteristics of and long-term outcome in Chinese patients with hypertrophic cardiomyopathy. METHODS: We evaluated 118 Chinese patients (62 male) who were diagnosed with hypertrophic cardiomyopathy at Queen Mary Hospital from 1973 to 2002. Diagnosis was based on the demonstration of left ventricular hypertrophy (wall thickness ≥15 mm during diastole), either in a specific region or with diffuse distribution, using echocardiography or magnetic resonance imaging. Clinical predictors of major cardiovascular events related to hypertrophic cardiomyopathy (cardiovascular death, potentially fatal cardiac arrhythmia, and refractory heart failure requiring cardiac transplantation or percutaneous alcohol septal ablation) were evaluated with univariate and multivariate Cox proportional hazards regression models. RESULTS: The mean (± SD) age at presentation was 54 ± 18 years. During a mean follow-up of 5.8 ± 4.3 years (range, 1 to 29 years) from presentation, major cardiovascular events related to hypertrophic cardiomyopathy occurred in 19 patients (16%), including 9 deaths. Annual cardiovascular mortality was 1.6%. Fifty-five patients (47%) had one or more cardiovascular complications related to hypertrophic cardiomyopathy, of which atrial fibrillation was the most common (35%, n = 41). The most common type of hypertrophic cardiomyopathy was the apical variant (41%, n = 49). In multivariate analysis, female sex was the only independent predictor of major cardiovascular events related to hypertrophic cardiomyopathy (hazard ratio = 5.86; 95% confidence interval: 1.77 to 7.21; P = 0.007). CONCLUSION: Hypertrophic cardiomyopathy in Chinese patients is characterized by late onset of presentation, a high incidence of the apical form of the condition, and adverse clinical outcome in female patients, which suggest a different phenotypical pattern than in white patients. © 2004 by Excerpta Medica Inc.link_to_subscribed_fulltex