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Published on: September 13, 2014
Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation
Matthew J Lewis1, Leigh C Reardon2, Jamil Aboulhosn2
1Division of Cardiology, Department of Medicine, Columbia University Medical Center, New York, New York, USA.
Insights
Fontan-associated liver disease (FALD) impacts survival after heart transplant (HT). Combined heart-liver transplant (CHLT) offers superior survival for Fontan patients with significant FALD, especially those with a FALD score of 2 or higher.
Area of Science:
- Cardiology
- Hepatology
- Transplantation Medicine
Background:
- Fontan-associated liver disease (FALD) poses unknown risks for adult Fontan patients undergoing heart transplant (HT).
- The comparative effectiveness of HT alone versus combined heart-liver transplant (CHLT) for these patients is not well-established.
Purpose of the Study:
- To evaluate the impact of FALD on post-transplant outcomes in adult Fontan patients.
- To compare survival rates between HT and CHLT in this population.
Main Methods:
- A retrospective cohort study included 131 adult Fontan patients (age ≥16) undergoing HT or CHLT across 15 centers.
- Pretransplant FALD score was determined by the presence of cirrhosis, varices, splenomegaly, or ≥2 paracenteses.
- Outcomes were compared between HT and CHLT groups, with subgroup analysis based on FALD score.
Main Results:
- CHLT recipients were older and had higher pretransplant FALD scores compared to HT recipients.
- CHLT showed a trend toward improved 1-year survival (93% vs 74%) and significantly improved 5-year survival (86% vs 52%) compared to HT alone.
- In patients with a FALD score ≥2, CHLT significantly improved 1-year (85% vs 62%) and 5-year (77% vs 42%) survival.
Conclusions:
- Higher FALD scores are linked to increased post-transplant mortality in Fontan patients.
- Combined heart-liver transplant (CHLT) demonstrates superior survival outcomes compared to heart transplant (HT) alone, particularly for Fontan patients with significant FALD (score ≥2).
- Further prospective studies are needed to confirm these findings.
Background:
The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown.
Objectives:
The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients.
Methods:
We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses.
Results:
A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]).
Conclusions:
Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.
