Advanced therapies for congenital heart disease: ventricular assist devices and heart transplantation

Improvement in pre-, peri-, and postoperative management of congenital heart disease (CHD) has significantly increased long-term survival in children with CHD. However, there is a subset of patients with CHD who are either poor candidates for surgical palliation or in whom surgical intervention has failed and require advanced cardiac support. Heart transplant (HT) as a therapy for CHD has undergone tremendous evolution. Though transplantation remains the standard of care to improve survival and quality of life when conventional medical and surgical therapies have failed, it remains limited by the scarcity and unpredictability of donor organ availability. As such, the use of ventricular assist devices (VADs) as a bridge to transplant is gaining increasing popularity. Because of improvement in device technology, and reduced rate of VAD-related complications, the use of these devices is expanding to smaller children and more complex congenital anatomy. Challenges with VAD support and HT in the congenital heart population will be addressed in this review with focus on: (1) reasons for VAD implantation; (2) VAD support in Fontan circulation; (3) challenges with human leukocyte antigen (HLA) sensitization in HT; and (4) effect of VAD support on HT in CHD.