SAN FRANCISCO, CA—Children with dilated cardiomyopathy have increased risk of transplantation and mortality, particularly within the first year of presentation, and may benefit from treatment with carvedilol and ACE inhibitors, a 14-year review of treating this patient population concluded at ACC.13, the American College of Cardiology’s 62nd Annual Scientific Session.

“Medical treatment in pediatric patients with dilated cardiomyopathy is not as well standardized as in the adult population in relation to medications and dosages, and use of carvedilol remains controversial,” noted Paolo G. Rusconi, MD, of the University of Miami Miller School of Medicine, Miami, FL, and colleagues.

They reviewed demographic, diagnosis, medication dosages, and echocardiogram measurements of ejection fraction, fractional shortening corrected by age (FSz), and left ventricular end diastolic diameter corrected by BSA to assess outcome and response to treatment among 106 children. Of the 106 children, 56 were male, and age at presentation was 7.4 ± 3.8 years.

A combination of carvedilol and an ACE inhibitor was used in 72% of children. The average dose in children <40kg of carvedilol was 0.95mg/kg/day (range 0.05–1.7), enalapril 0.64mg/kg/day (0.08–1.2) and captopril 3.4mg/kg/day (0.45–6.4). Patients >40kg received standard adult doses. Average follow up was 6.0 ± 5.4 years.

“Death or transplant occurred in 47% of children,” Dr. Rusconi noted. In the survivors, ejection fraction improved 34.3 ± 13.9 to 53.2 ± 10.1 (P<0.001) and fractional shortening corrected by age from -7.1 ± 6.6 to -3.0 ± 3.3 (P<0.001)

Left ventricular end diastolic diameter corrected by BSA decreased from 3.9 ± 2.7 to 1.8±2.1 (P=0.02). Only three patients did not tolerate carvedilol—two due to asthma and one due to worsening heart failure. Also, three patients each experienced one episode of hypoglycemia.

“Carvedilol and ACE inhibitors are well tolerated at the dose used and appear to be safe when used long-term,” he concluded. “However, efficacy still remains to be demonstrated.”