Guideline-directed medical therapy for dilated cardiomyopathy in the era of pediatric ventricular assist devices: Improving transplant-free survival

Abstract

Background

Cardiomyopathy remains the leading cause of transplantation in children greater than one year of age, with low five-year transplant free survival. Despite these poor outcomes, the potential for myocardial recovery in pediatric dilated cardiomyopathy (DCM) has long been reported. In the current era of guideline directed medical therapy (GDMT), however, achievement of myocardial recovery has not been rigorously studied. Ventricular assist devices (VADs) can also be employed as a bridge to recovery, but VAD explants for recovery are rare.

Aims of review

A focus of the Sixth International Conference on Childhood Cardiomyopathy was achievement of myocardial recovery in the era of GDMT and VADs. Key questions included: (1) what is the potential for myocardial recovery; (2) what role does GDMT play in achieving myocardial recovery; (3) can durable left ventricular assist devices (LVADs) be a reasonable means to achieving recovery; (4) how do rates of recovery compare between GDMT and LVADs; and (5) what are the next steps in improving rates of myocardial recovery? This review aims to answer these questions by providing a summary of the existing literature.

Key scientific concepts of review

The future directions for pediatric DCM are to use GDMT and VADs as complementary therapies to improve not only survival to transplant, but to assess for recovery parameters so that more can be learned about the potential for transplant-free survival in patients supported by VAD and GDMT.