Transplantation and Mechanical Circulatory Support in Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Abstract

Dramatic evolution in the medical and surgical care of children with congenital heart disease (CHD) has led to a growing number of adults with late-onset complications, including heart failure (HF).1 In parallel with an overall increase in hospital admissions for adults with CHD (ACHD) and HF,2 CHD complexity has increased substantially in survivors over the past 2 decades.3 Heart transplant (HTx) specialists face the challenge of determining eligibility for advanced HF treatments among an increasingly complex population of CHD patients in whom guidelines for HTx and mechanical circulatory support (MCS) are scant. The purpose of this review is to provide a state-of-the-art update on HTx and MCS in CHD. HTx remains the surgical procedure of choice for eligible patients with severe advanced HF,4,5 with little change in the number of transplants performed yearly over the past decade. The body of information related to transplantation for CHD is derived almost entirely from registry and single-center–based outcome data; no randomized clinical trial or meta-analysis data are available. CHD presents additional challenges to successful HTx compared with HTx in patients with acquired HF. Many CHD patients require complex vascular reconstruction at the time of transplantation. The presence of antibodies to human leukocyte antigen (HLA) and ABO blood group sensitization are also impediments to timely transplantation. The ability of patients with single-ventricle physiology to survive during the waiting period is also limited by the additional burden of “outgrowing” their pulmonary blood flow and the resultant cyanosis. It is not surprising that CHD remains a risk factor during the waiting period and after transplantation. Therefore, although the management of the CHD patient with end-stage HF must include the option of HTx, its indication and timing are very different from that for acquired HF. Patients with ACHD represent an increasing …