2006 Volume 70 Issue 12 Pages 1543-1549
Background The purpose of the present study was to assess the clinical long-term course of hypertrophic cardiomyopathy (HCM) in a rural Japanese cohort. Methods and Results A total of 137 consecutive HCM patients (mean age at diagnosis: 52±13 years) were enrolled. During a follow-up period of 11.4±5.7 years, 28 patients died of HCM-related causes. Eleven patients died suddenly, 10 died of progressive heart failure, 6 died of stroke associated with atrial fibrillation and 1 died of a postoperative complication of alcohol septal ablation. For the overall study group, 5-, 10- and 15-year cumulative survival rates were 91%, 88% and 79%, respectively. Although sudden death was the predominant cause of HCM-related death during the follow-up period of <10 years, heart failure death increased after follow-up period of >10 years. Fifteen (13%) of 114 patients who had follow-up echocardiography became `end-stage' HCM and 8 patients died of severe and refractory heart failure. They already showed minimally dilated left ventricular (LV) dimension and lower LV fractional shortening at initial evaluation. Conclusions Although HCM patients in a Japanese rural community showed relatively benign clinical course (the same as cohorts in the developed world), heart failure death because of LV remodeling became equally important to sudden death when they were followed for more than 10 years. (Circ J 2006; 70: 1543 - 1549)
