Multi-Organ Transplantation in Adult Congenital Heart Disease: Navigating the Unique Challenges of a Distinct Patient Population

Abstract

The prevalence of adult congenital heart disease (ACHD) is increasing, with heart failure being the leading cause of death. For many ACHD patients, heart transplantation is the only treatment option for advanced heart failure, though significant extracardiac involvement may require multi-organ transplantation. Despite the rising number of ACHD transplants, multi-organ transplants in this population remain challenging, and a substantial gap remains between those in need and those who receive a transplant. While short-term outcomes may be worse for ACHD patients, long-term outcomes are comparable and even superior to other cardiomyopathies. Extracardiac organ dysfunction is common in ACHD patients, often precluding heart-alone transplantation. Fontan-associated liver disease, pulmonary vascular and restrictive lung disease, and renal dysfunction frequently necessitate multi-organ transplantation. ACHD patients have a unique immunological and sensitization profile, increasing their risk for infection, rejection, and malignancies, requiring specialized pretransplant desensitization and post-transplant immunosuppression strategies. ACHD transplantation presents unique surgical challenges, including chest reentry, vascular access issues, bleeding risks, extensive anatomical reconstruction, the need for longer vascular segments from donors, and prolonged ischemic times. Decisions regarding heart-alone versus heart-liver, heart-lung, or heart-kidney transplantation demand careful evaluation. These complex surgical plans require extensive multimodal imaging and collaboration with ACHD cardiac imaging specialists and abdominal transplant teams. Comprehensive coordination and psychosocial support are crucial for ACHD patients throughout the transplant process. A dedicated multidisciplinary team and an established and separate pathway for pre-, peri-, and postoperative care in centers with ACHD and multi-organ transplant expertise are essential. There is need for a revised organ allocation system to ensure timely access to transplantation for ACHD patients.

Keywords: adult congenital heart disease; complication associated with adult congenital heart disease; heart transplant; multi-organ transplant.

Figure 1

Cardiac magnetic resonance demonstrating right ventricle and main pulmonary artery adhesions to the sternum in a patient with double outlet right ventricle status post biventricular repair with arterial switch operation. S: sternum, RV: right ventricle, MPA: main pulmonary artery

Figure 2

Computed tomography angiography of the chest with extensive collateral networks around the airway, the SVC and the aorta in a patient with unbalanced complete atrioventricular canal status post Fontan palliation. (*) Collateral networks, AAo: ascending aorta; Dao: descending aorta; SVC: superior vena cava

Figure 3

Patient with left superior vena cava (LSVC) and right superior vena cava (RSVC) who needs reconstruction for preparation for donor superior vena cava.

Figure 4

Patient who needs reconstruction of pulmonary arteries with a patch (*) to create a landing zone (*) for the donor pulmonary arteries.