Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function

Abstract

Background Liver cirrhosis is recognized with long-term follow-up of patients after the Fontan procedure. The effect of liver cirrhosis on the use of heart transplant (HT) and on post-HT outcomes is unknown. Methods We reviewed Fontan patients evaluated for HT from 2004 to 2012 with hepatic computed tomography (CT) imaging, classified as normal, non-cirrhotic changes, or cirrhosis. The primary outcome was 1-year all-cause mortality, and the secondary outcome was differences in serial post-HT liver evaluation. Results CT imaging in 32 Fontan patients evaluated for HT revealed 20 (63%) with evidence of liver disease, including 13 (41%) with cirrhosis. Twenty underwent HT, including 5 non-cirrhotic and 7 cirrhosis patients. Characteristics at listing between normal or non-cirrhotic (n = 13) and cirrhosis (n = 7) groups were similar, except cirrhosis patients were older (median 17.6 vs 9.6 years, p = 0.002) and further from Fontan (median 180 vs 50 months, p < 0.05). Serial liver evaluation was similar, including aspartate aminotransferase, alanine aminotransferase, bilirubin, albumin, and tacrolimus dose at 1, 3, 6, 9, and 12 months. Overall patient survival was 80% at 1 year, with no difference between cirrhosis and non-cirrhosis patients (86% vs 77%, p = 0.681). Liver biopsies were performed in 7 patients before HT, and all specimens showed architectural changes with bridging fibrosis. Conclusions Most patients evaluated for HT had abnormal liver findings by CT, with cirrhosis in 41%. One-year mortality and serial liver evaluation were similar between groups after HT. Liver cirrhosis identified by CT imaging may not be an absolute contraindication to HT alone in this population.

Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes

“…Another recent study found no association between hepatic imaging and post-transplant outcomes in Fontan patients. 30 Therefore, we recommend considering histopathologic findings as one part of the greater picture when evaluating subjects for transplant.…”

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

“…In multivariate analysis, only the time since initial Fontan procedure was associated with hepatic complications. Importantly, Simpson et al 151 found in a small study of Fontan patients (mean age, 13 years) that computed tomography evidence of liver cirrhosis did not predict 1-year mortality after HTx. They did not systematically assess MELD scores, histology, or HVPG.…”

Changing Indications for Pediatric Heart Transplantation

“…100,101 The impacts of portal fibrosis and cirrhosis on outcomes after transplantation are under investigation, with a few patients undergoing heart and liver transplantation when advanced cirrhosis is present. [102][103][104] The type of congenital heart disease also affects survival after transplantation. Patients who have undergone the Fontan procedure have a higher posttransplantation mortality than patients with other types of congenital heart disease, with one series reporting a hazard ratio of 8 for early mortality.…”