When to refer adult patients with congenital heart disease for transplantation: Which criteria to use, which work-up?

Being the most common mode of death, heart failure related to adult congenital heart disease (ACHD-HF) will be one of the main challenges for the ACHD community in the coming years. With increasing number of ACHD-HF patients and an increase in ACHD-HF related admissions, there is an increasing demand for advanced HF therapies such as the use of ventricular assist devices and transplantation. Unfamiliarity with the underlying anatomy and pathophysiology, a higher peri-operative transplant mortality and the absence of specific transplant indication criteria put our patients at a disadvantage, making them less attractive candidates for transplant listing. The development of an advanced ACHD-HF service as part of a larger transplant program is a logical/necessary/mandatory next step to optimize and guide clinical care and increase access to advanced HF therapies for ACHD-HF patients. Ultimately increasing knowledge and building evidence should result in better quality of care and improved outcomes for ACHD-HF patients. The purpose of this review article is to provide a sense on how we are evaluating ACHD-HF patients, identifying those who may benefit from advanced HF therapy and eventually deciding on who should be listed for transplant.