Title : Mechanical circulatory support and transplantation in adult congenital heart disease

As the adult  population with congenital heart disease (ACHD) continues to grow due to the improved survival in pediatric cardiac surgery, heart failure in this group of patients has become an increasing problem in the last two decades. If the conservative treatment fails, mechanical circulatory support (MCS) and heart transplantation need to be considered. The use of VAD has become more popular in ACHDand the increasing experience has made the VAD a suitable option in select patients, with sometimes limited possibilities particularly in the single ventricle patients. Heart Transplant (HTx) is a suitable option for ACHD patients who have severe heart failure and Transplant outcomes overall improved in current era. Congenital heart disease diagnosis does increase risk of early mortality but not late mortality in adult congenital population, and the overall survival in ACHD population is quite good with HTx , and HTx should not be denied as a form of therapy in ACHD. As adult congenital population increases, we need to consider modifying listing status criteria in many countries to accommodate these patients, because, at the moment, they are in disadvantage in comparison to the standard adult population with end-stage heart failure, with higher wait list mortality. Future directions should be concentrated on research for better mechanical support options particularly in single ventricle patients, formalized transition programs from pediatric cardiology to ACHD cardiology, centralizing care to specialized ACHD centers, particularly for those with heart failure and increasing attention given to the hepatic function in Fontan patients. This subgroup of ACHD should have a regular careful assessment of liver function, special imaging and hepatologists always consulted and part of decision making for heart transplant only or heart-liver transplant.

Keywords: Heart Transplant - adult -  - congenital – heart disease – Fontan – liver

Cardio-Thoracic Surgeon with 25 years of experience in congenital heart disease and thoracic transplantation, expert in mechanical assist devices and ECMO. Especially focused on mechanical support and transplantation for single ventricle circulation.