Key result
Primary cardiomyopathy affects ~2 per 100,000 Korean children, primarily presenting as dilated cardiomyopathy.
Why the study?
Epidemiologic data on idiopathic cardiomyopathies have become very important with advances in therapeutic strategies, but only a few have been reported in Asian children.
Observational (n=277)
Yes
The point prevalence of childhood primary cardiomyopathy in Korea is 2.11/100,000, with varying survival rates depending on the subtype.
Informs Korean pediatric cardiology resource planning; extends observational epidemiology to East Asian children while leaving generalizability open.
BACKGROUND: Idiopathic cardiomyopathies (CMPs) are an important heterogeneous group of diseases. With the advance of therapeutic strategies, epidemiologic data on CMP have become very important, but only a few have been reported in Asian children. We conducted a retrospective epidemiologic study of primary CMP in Korean children. METHODS AND RESULTS: Using a multicenter survey, we studied primary CMP among Korean children from January 1998 to December 2006 based on classification (2006) of CMP by the American Heart Association. A total of 277 primary CMP patients were reported from 17 cardiovascular centers. The average annual occurrence of new cases of primary CMP was 0.28 per 100,000 Korean children younger than 15 years of age (95% confidence interval (CI) 0.24-0.31). Dilated CMP (DCMP) was 66.43%, hypertrophic CMP (HCMP) 23.47%, restrictive CMP (RCMP) 6.50% and others 3.61%. The point prevalence of primary CMP at the end of the study was estimated as 2.11/100,000 (95%CI 1.83-2.43), DCMP 1.39/100,000, HCMP 0.51/100,000, RCMP 0.16/100,000 and others 0.04/100,000. Survival rates over 9 years were 69.8% in DCMP, 90.3% in HCMP, and 47.2% in RCMP. CONCLUSIONS: Recent point prevalence of childhood primary CMP in Korea was estimated as 2.11/100,000. Further epidemiologic study with a nationwide survey is necessary.
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Oh et al. (2011) conducted an observational in Primary cardiomyopathy (n=277). Primary cardiomyopathy was evaluated on Point prevalence of primary cardiomyopathy per 100,000 children (95% CI 1.83-2.43). The point prevalence of primary cardiomyopathy in Korean children under 15 years of age was estimated at 2.11 per 100,000, with dilated cardiomyopathy being the most common subtype.
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