Initial experience with combined heart-liver transplantation in the Netherlands: Exploring the boundaries of isolated and combined transplantation

Abstract

Heart transplantation is considered as the ultimate treatment for patients with advanced heart failure (HF). Chronic HF is associated with hepatic congestion and reduced cardiac output, which can lead to progressive liver disease. This issue is particularly relevant in patients with congenital heart disease, especially those with a single functional ventricle managed through Fontan-type surgery. The presence of advanced liver disease may contraindicate isolated heart transplantation and thus require consideration of combined heart-liver transplantation (CHLT). However, consensus criteria for CHLT have not yet been established. To illustrate the clinical and scientific discussions on this topic, we present the clinical course of two patients with a Fontan circulation who were evaluated for CHLT and discuss decision-making factors based on a review of current literature. We conclude that establishing a CHLT program represents a promising therapeutic pathway for patients in the Netherlands with advanced HF and concomitant liver disease. Both isolated heart transplantation and CHLT are viable treatment approaches for carefully selected patients with HF and liver disease. However, early identification of potential candidates and timely referral for a comprehensive evaluation are essential for the effective management of this high-risk patient group.

Keywords: Congenital heart disease; Fontan failure; Fontan-associated liver disease; Heart transplantation; Heart-liver transplantation.

Fig. 1

Infographic

Fig. 2

Panel a shows the anatomic situation in patient 1 with lateral tunnel Fontan circulation. Panel b pericardial view after completed cardiectomy and patch reconstruction of the right pulmonary artery in preparation for orthotopic heart transplantation. Panel c depicts the end result after a combined heart-liver transplantation

Fig. 3

Panel a total number of heart transplants in the Eurotransplant region (Austria, Belgium, Croatia, Germany, Hungary, Luxembourg, the Netherlands and Slovenia). Panel b total number of heart liver transplants in the Eurotransplant region in actual numbers (bars) and as a percentage of all heart transplants (line). Data obtained from Eurotransplant. Panel c total number of heart transplants in the in United States. Panel d total number of heart liver transplants in the Eurotransplant region in actual numbers (bars) and as a percentage of all heart transplants (line). Please note that the y‑axis scale differs from that in Panel b. Data obtained from the Organ Procurement and Transplantation Network

Fig. 4

Flowchart for liver assessment in patients with congenital heart disease/Fontan physiology to be considered for isolated heart transplant or combined heart-liver transplant. Text in Italic includes evaluated patients who received/were enlisted for combined heart-liver transplant (CHLT) or isolated heart transplant (HTx). Patients without extensive fibrosis or collaterals are not included in this flowchart. *Hepatic decompensation includes severe synthetic dysfunction of the liver, hepatic ascites (low protein, high liver-ascites albumin gradient), hepatic encephalopathy, and recurrent portal hypertensive bleeding. FALD Fontan Associated Liver Disease, AFP alpha-fetoprotein