Variant R94C in TNNT2‐encoded troponin T predisposes to pediatric restrictive dardiomyopathy and sudden death through impaired thin filament relaxation resulting in myocardial diastolic dysfunction

Background Pediatric-onset restrictive cardiomyopathy (RCM) is associated with high mortality, but underlying mechanisms of disease are under investigated. RCM-associated diastolic dysfunction secondary to variants i

Mechanical Circulatory Support in Pediatric and Adult Congenital Heart Disease.

The use of ventricular-assist device (VAD) support continues to increase and it is now considered part of the standard of care for most adult and children with end-stage heart failure unresponsive to medical management. However, the application of VAD therapy in both the pediatric and adult population with congenital heart disease (CHD) has been less extensive and continues to face significant challenges. Nevertheless, the experience is growing and the use of VADs in the CHD population is no longer limited to case reports, thereby increasing our understanding of how best to support this diverse patient population. The current body of literature provides a framework to understand the outcomes of this unique patient population with respect to mortality, risk factors, and associated morbidities in both adult and pediatric patients with CHD. This review will summarize that body of literature with focus on outcomes based on device type and anatomy. We also highlight efforts made by the community to improve outcomes of patients with CHD on VAD support