Dilated cardiomyopathy in children

Abstract

Dilated cardiomyopathy (DCM) is a rare myocardial disorder characterized by a dilated left ventricle and systolic dysfunction. Globally, it affects around 1 in every 100 000 children. The prognosis is generally poor, with 40% either failing traditional medical therapy within the first 2 years or requiring a heart transplant. This article will address the basic cause, epidemiology, pathobiology, and historical treatment approach of DCM and introduce novel contemporary medical and surgical strategies that may reduce the need for heart transplantation. In the last 15 years, there has been a significant emphasis on identifying alternative treatment strategies in managing the child with a DCM and heart failure symptoms. New therapies have evolved to help bridge these critically ill children to transplant or have these therapeutic modalities serve as end-points in themselves. Thus subsequently, we will highlight contemporary as well as novel medical and procedural therapies that are being used for the treatment of pediatric DCM. The child with a DCM and severe left ventricular dysfunction has a number of options available beyond simple diuretics and afterload reduction. Novel pacing strategies and mechanical assist device may provide not only a more stable clinical bridge environment but also may actually serve as an endpoint itself.