Research Summary

AHA Scientific Statement Focuses on Treatment Strategies for Pediatric Cardiomyopathy

Jessica Bard

A scientific statement from the American Heart Association addresses treatment strategies for cardiomyopathy in children based on three core principles to further refine and improve the health and outcomes of this patient population.1

The core principles include identifying cardiac pathophysiology, understanding the cause of the cardiomyopathy, and applying the therapies based on the patient’s associated clinical milieu, such as risk for developing cardiomyopathy, patients who are asymptomatic with cardiomyopathy, patients with symptomatic cardiomyopathy, or patients with end-stage cardiomyopathy.

“This scientific statement focuses primarily on the most frequent phenotypes, dilated and hypertrophic, that occur in children,” according to the researchers. “Other less frequent cardiomyopathies, including left ventricular noncompaction, restrictive cardiomyopathy, and arrhythmogenic cardiomyopathy, are discussed in less detail.”

The recommendations are based on the differences in pathogenesis and pathophysiology in cardiomyopathies in children compared with adults and will likely affect the applicability of some adult therapies. 

The statement suggests several key points: 1) initiate treatment in some children who are at risk for cardiomyopathy before symptoms occur to slow the disease progression; 2) adult heart failure treatment has contributed to fewer deaths among children with cardiomyopathy, although the treatments have limited effects; 3) therapies used in children with hypertrophic cardiomyopathies to prevent sudden death continue to evolve and are specific to age and size, compared with adults; 4) a heart transplant is an acceptable treatment for end-stage pediatric cardiomyopathy.

The researchers wrote this statement to supplement an earlier statement on the classification and diagnosis of cardiomyopathy in children.2

References:

  1. Bogle C, Colan SD, Miyamoto SD, et al; American Heart Association Young Hearts Pediatric Heart Failure and Transplantation Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young (Young Hearts). Treatment strategies for cardiomyopathy in children: A scientific statement from the American Heart Association. Circulation. 2023;148(2):174-195. doi:10.1161/CIR.0000000000001151
  2. Lipshultz SE, Law YM, Asante-Korang A, et al. Cardiomyopathy in children: classification and diagnosis: A scientific statement from the American Heart Association. Circulation. 2019;140(1):e9-e68. doi:10.1161/CIR.0000000000000682