When Should Adult Congenital Heart Disease Patients be Considered for Transplant and Deciding Which Organs to Transplant

The number of adult congenital heart disease (ACHD) patients continues to increase. Because of multiple related factors such as aging, residual cardiac lesions and prior palliative procedures, advanced heart failure (HF) is increasingly prevalent in this population. Consequently, there is an emerging need to determine which patients are best suited for advanced cardiac therapies, including heart transplantation (HT) and mechanical circulatory support. Unfortunately, optimizing patient selection for these therapies is complicated by patient heterogeneity, variable HF presentation across lesion-type, and a paucity of outcome data. The lack of patient specific data and the increasing number of ACHD patients with end-stage HF identifies a need to more precisely stratify risk and determine appropriate timing for referral. As such, this article will discuss the clinical recognition and classification of advanced HF in the ACHD patient populations and review current data regarding HT outcomes. Following, key considerations regarding the timing of HT in specific forms of ACHD, the role of device therapy, and when dual organ transplantation should be considered will be reviewed. Finally, existing knowledge gaps and key research needs will be highlighted.