Idiopathic Cardiomyopathies in Korean Children - 9-Year Korean Multicenter Study -

Abstract

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. (Circ J 2011; 75: 2228 - 2234

Childhood Cardiomyopathies: A Study in Tertiary Care Hospital in Upper Egypt

“…The mortality rate was 10% of cases at the end of our study period. Our results are close to that of Towbin’s study where the 1-year rate of death was 13% (14), and better than that of a Korean study (19.8% at the end of the first year), however, results could not be compared to as long a term as the Korean study (death rate was 26.7% after eight years follow up) (12). …”

“…None of the cases of our study has undergone cardiac transplantation, ventricular assist device (VAD), intra-aortic balloon pump (IABP), or connected to ECMO. In a similar Korean study, 3% of patients underwent cardiac transplantation, 1% had a VAD, and 0.3% had IABP (12). In an American study, approximately 5% of patients underwent cardiac transplantation, 2% IABP, 2% ECMO, and 1% VAD (17).…”

“…According to the United States (US) Pediatric Cardiomyopathy Registry, DCMP was the most common (58%), followed by HCMP (30%), RCMP (5%) and ARVC (5%) (4). In a study on Korean children, DCMP was (66.43%), HCMP (23.47%), RCMP (6.50%), and others (3.61%) (12). All these studies coincide with the results of our study in that, the most common type of CMP is DCMP followed by HCMP.…”

“…The overall long-term prognosis of pediatric patients depends on the CMP type, and disease stage (3). The 9-year survival rate was estimated to be 69.8% in DCMP, 90.3% in HCMP, 47.2% in RCMP, and 42.0% in other CMP patients (12). The objectives of the present study were to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital.…”

Massive Cardiomegaly due to Dilated Cardiomyopathy Causing Bronchial Obstruction in an Infant

“…Despite recent advances in cardiopulmonary support strategies, dilated cardiomyopathy (DCMP) remains a life threatening disease in young patients 1)2). Early recognition and treatment of DCMP are crucial for good prognoses in this patient population 3)4).…”