Long-term Outcomes Suboptimal for Children With Hypertrophic Cardiomyopathy

A study of 100 children with hypertrophic cardiomyopathy (HCM) found long-term outcomes to be suboptimal, and in those with secondary HCM to be even worse. Researchers published their findings in Journal of Cardiology.

Children in the study were patients in the National Cerebral and Cardiovascular Center Department of Pediatric Cardiology in Osaka, Japan, between 1977 and 2015. The median age of diagnosis was 8 years, and 54 patients were male and 46 were female. Thirteen patients had Noonan syndrome, 13 had hypertrophic obstructive cardiomyopathy, 65 had idiopathic HCM, and 9 had secondary HCM.

A medical record review revealed that 24 patients experienced a dilated phase of HCM. Among them, 19 patients died of heart failure, and 2 received a heart transplant. The median age when a dilated phase occurred was 15 years, and the median time from diagnosis of a dilated phase to patients dying was 1.6 years.

At around 15 years of age, 6 patients died suddenly, and cardioverter defibrillator implantations occurred in 11 patients, according to study authors.

Overall survival rates were at 83% at 10 years, 69% at 20 years, and 63% at 30 years. Overall cardiac-event-free survival rates were 57% at 10 years, 39% at 20 years, and 32% at 30 years, reported researchers.

Per diagnosis, 20-year survival rates were 84% with Noonan syndrome, 82% with hypertrophic obstructive cardiomyopathy, 71% with idiopathic HCM, and 17% with secondary HCM, study authors found.

Reference:
Tsuda E, Ito Y, Kato Y, Sakaguchi H, Ohuchi H, Kurosaki K. Thirty-year outcome in children with hypertrophic cardiomyopathy based on the type. J Cardiol. 2022;S0914-5087(22)00194-0. doi:10.1016/j.jjcc.2022.07.016