Outcomes of Heart and Combined Heart-Liver Transplant in Pediatric Fontan Patients

Abstract

While combined heart-liver transplant (HLT) is being performed for Fontan patients, outcomes and indications remain poorly defined. We compared listing characteristics and post-transplant outcomes of Fontan patients with HLT to those with heart-only transplant (HT). We retrospectively reviewed all Fontan patients undergoing HT or HLT at our institution between 2006-2019. Pre-transplant liver disease at the time of listing, as determined by laboratory and radiology data, and MELD-XI, Child Pugh, and VAST scores, were compared between groups. The Kaplan Meier survival method was used to compare post-transplant survival and freedom from rejection (ISHLT Grade 2R+ or pAMR2+). Forty-two patients (33 HT, 9 HLT) were included. HLT patients were older, more likely to be on dual-inotrope therapy, had worse Child Pugh and VAST scores, and were more likely to have varices, ascites, splenomegaly, and cirrhosis on imaging (Table 1). While not significant, HLT patients tended to be more sensitized, had higher total bilirubin and creatinine, and lower platelet count. Over a median post-transplant follow-up of 22 (5, 62) months, overall mortality for the entire cohort was 19%; only one HLT patient died (11%) versus 7 (21%) HT patients (p=0.58, Figure 1). None of the HLT patients experienced rejection, whereas 11 (33%) HT patients had rejection (p=0.06). Despite a greater inotrope need and more severe liver disease at time of listing, patients undergoing HLT have post-transplant outcomes that are comparable, if not better, than Fontan patients with HT. There appears to be a protective effect from rejection with HLT.