Heart transplant for pediatric cardiomyopathy

Abstract

Heart transplantation is usually utilized in pediatric patients with dilated or restrictive cardiomyopathies, or in patients with hypertrophic cardiomyopathies with the hemodynamic characteristics of dilated or restrictive cardiomyopathies. Pediatric Cardiomyopathy Registry (PCMR) data suggests that transplantation is utilized in patients with idiopathic, familial, or myocarditis dilated cardiomyopathies to a greater extent than in dilated cardiomyopathies observed in malformation syndromes, inborn errors of metabolism or neuromuscular disease. Single and multicenter studies suggest that lower left ventricular ejection fraction at presentation is associated with a greater likelihood and a diagnosis of myocarditis to a decreased likelihood of heart transplantation respectively. International Society for Heart and Lung Transplantation (ISHLT) data demonstrates cardiomyopathy is making up an increasing proportion of heart transplantation in infants. PCMR data suggest that the overall freedom from death or transplantation in patients with pediatric dilated cardiomyopathy has changed little in the past 15 years. However, survival after pediatric heart transplantation has improved over the same time period. Two single center studies have found that while survival with pediatric dilated cardiomyopathy has improved, the freedom from death or transplantation is similar to survival in pediatric dilated cardiomyopathy prior to the routine use of heart transplantation. Furthermore, these studies could find no impact from new medical therapies on transplant-free survival. These findings suggest that heart transplantation may be the most effective therapy for improved survival in pediatric dilated cardiomyopathy.