PURPOSE: Hypertrophic cardiomyopathy (HCMP) is a primary cardiac muscular disease with high risk of sudden cardiac death. This study is performed to understand the clinical features of children with HCMP. METHODS: Retrospective analysis of the medical records of the patients diagnosed at Seoul National University Children's Hospital between October 1985 and June 1996 was done. Excluded the patients who had been lost to followed up within 12 months after diagnosis. RESULTS: During follow-up (mean 56 months, range 1 month to 13 years), 3 of the 24 patients (male 15 and female 9, mean age at diagnosis 5.9 years, range 2 months-15 years) died suddenly and 1 died of persistent heart failure after cardiac resuscitation. Cumulative death rate for 56 months was 22.3%. Eighteen were treated with -blocker including 1 with additional amiodarone. Normal quality of life except competetive exercise were maintained in the 95% of the survivors. In this study, moderate to severe dyspnea on exertion (New York Heart Association III/IV) was the only significant risk factors of death (p<0.05). On the surface electrocardiogram, QTc interval was significantly longer in the study group comparing with the age and sex matched control group. Right ventricular outflow obstruction was associated in the 3 (12%) and was common in infants (33%). CONCLUSIONS: Overall mortality is high in children with HCMP. In our study. cumulative death rate for 56 months is 22.3%. Overt dyspnea on exertion was the only identifiable factor of mortality.
Amiodarone ; Cardiomyopathy, Hypertrophic* ; Child* ; Death, Sudden, Cardiac ; Diagnosis ; Dyspnea ; Electrocardiography ; Female ; Follow-Up Studies* ; Heart ; Heart Failure ; Humans ; Infant ; Medical Records ; Mortality ; Muscular Diseases ; Quality of Life ; Resuscitation ; Retrospective Studies ; Risk Factors ; Seoul ; Survivors ; Ventricular Outflow Obstruction