Heart Transplant vs. Combined Heart-Liver Transplant in Adult Fontan Patients

Quick Takes

  • After matching for baseline characteristics, combined heart-liver transplant was associated with improved survival at 5 years as compared with heart transplant alone.
  • In patients with a Fontan-associated liver disease (FALD) score ≥2, combined heart-liver transplant was associated with improved survival at both 1 and 5 years. For the FALD score, 1 point was given for each of the following: cirrhosis by imaging or liver biopsy, varices, splenomegaly, or the need for ≥2 paracenteses.
  • Given significant limitations related to the retrospective nature of the study and criteria for cirrhosis, prospective studies will ultimately be required to better understand which patients may be candidates for heart transplant alone as compared with combined heart-liver transplantation.

Study Questions:

What is the impact of Fontan-associated liver disease (FALD) on post-transplant outcomes in adult Fontan patients, and is there a benefit of combined heart-liver transplant (CHLT) over heart transplant (HT) alone?


A multicenter retrospective cohort study was performed. Inclusion criteria included Fontan circulation, HT/CHLT referral, and age ≥16 years at time of referral. Pre-transplant FALD score was calculated based on presence of cirrhosis by imaging or biopsy, varices, splenomegaly, or the need for ≥2 paracenteses. One point was given for each of these factors present. Propensity-score matching and subgroup analyses were performed.


A total of 131 patients were studied, of which 91 patients underwent HT alone and 40 underwent CHLT. CHLT recipients were more likely to be older, have a lower hemoglobin, require ≥2 diuretics prior to transplant, or be transplanted in more recent decades. Post matching, CHLT demonstrated a trend towards improved survival at 1 year (74% vs. 93%, p = 0.097) and improved survival at 5 years (52% vs. 86%, p = 0.41) compared to HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 62% vs. 85%, p = 0.044; 5 years: 42% vs. 77%, p = 0.019).


Higher FALD scores were associated with post-transplant mortality. This retrospective, nonrandomized study suggested that CHLT recipients were older with higher FALD scores, but had similar survival overall, and superior survival in patients with a FALD score ≥2.


Optimal timing of cardiac transplantation, as well as understanding which patients benefit from CHLT as opposed to HT alone are among the most difficult issues faced by adult congenital cardiology practitioners. This retrospective review examined the outcomes for Fontan patients undergoing both HT and CHLT. Patients with more advanced liver disease appeared more likely to benefit from combined transplant. Despite propensity-score matching, a possibility of bias remains. A significant limitation of the study as acknowledged by the authors is the difficulty in defining cirrhosis in this patient population. Some studies have reported nearly universal presence of cirrhosis in patients with Fontan circulation by liver biopsy, and there can be a fair amount of variability in interpretation of imaging studies. Further study will require a more standardized definition of cirrhosis.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Acute Heart Failure, Heart Transplant, Interventions and Imaging, Interventions and Structural Heart Disease

Keywords: Biopsy, Diagnostic Imaging, Diuretics, Fontan Procedure, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Hemoglobins, Liver Cirrhosis, Liver Transplantation, Paracentesis, Splenomegaly, Survival

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