What is the clinical evidence from this study?

Study design: Cohort. Population: Hypertrophic cardiomyopathy (n=137). Intervention: Longitudinal observation. Primary outcome: HCM-related death (sudden death, heart failure death, stroke death).

January 1, 2006Open Access

Left Ventricular Remodeling of Hypertrophic Cardiomyopathy Longitudinal Observation in a Rural Community

Key Result

In a rural Japanese cohort of patients with hypertrophic cardiomyopathy, 28 of 137 patients died of HCM-related causes over 11.4 years, with heart failure death becoming predominant after 10 years.

Study Design

Type

Cohort (n=137)

Multicenter

Structured PICO

Population

137 consecutive patients with hypertrophic cardiomyopathy (HCM) in a rural Japanese community (Kochi prefecture). Mean age 52±13 years, 68% male. Key inclusion: echocardiographic demonstration of hypertrophied left ventricle (wall thickness ≥15mm) without hypertension and other cardiac diseases.

Outcome

Cardiovascular death (composite of sudden death, heart failure death, and stroke death) and left ventricular remodeling over long-term follow-uphard clinical

In a rural Japanese cohort with hypertrophic cardiomyopathy, heart failure death due to left ventricular remodeling becomes as prominent as sudden death after 10 years of follow-up.

Limitations

The cohort size was relatively small and the design was retrospective
The patient cohort differs from other reports, being older and having less prevalence of LV outflow gradient
No significant difference in previously reported prognostic risk factors of sudden death between survivors and those who died suddenly
Patient management (lack of surgical treatment or ICD for primary prevention) could have affected the results

Abstract

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. 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.

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