Combined Heart-Lung-Liver Transplantation for Patients With Cystic Fibrosis: The Australian Experience

Transplant Proc. 2021 Sep;53(7):2382-2389. doi: 10.1016/j.transproceed.2021.07.028. Epub 2021 Aug 16.

Abstract

Background: Combined multivisceral transplantation has emerged as a therapeutic option for a select patient cohort; however, clinical decision-making remains complex and controversial. The aim of this study was to examine patient characteristics, operative complications, and long-term outcomes of all patients who have undergone combined heart-lung-liver transplantation (HLLTx) in Australia.

Methods: In this study, we performed a retrospective analysis of all adult patients who have undergone combined HLLTx in Australia to date. Recipient clinical characteristics, waitlist, and transplant outcomes are described.

Results: Eight adult patients have received HLLTx at a single Australian transplant center. Recipients of HLLTx have typically been young (median age, 30.1 years; range, 24-37), underweight (median body mass index, 19.8 kg/m2; range, 16.2-30.4) patients with cystic fibrosis (n = 8, 100%) with severe airflow obstruction (median forced expiratory volume in the first second of expiration, 24% predicted; range, 17%-48%) accompanied by liver cirrhosis confirmed on histopathology (n = 8, 100%). Despite relative preservation of synthetic function and low model for end-stage liver disease scores (median, 8; range, 6-17), all recipients had complications of portal hypertension prior to transplantation, with many patients having suffered life-threatening variceal hemorrhage. In this cohort, HLLTx was associated with overall posttransplant survival of 87.5% at 30 days, 71.4% at 1 year, and 42.9% at 5 years. Listing for combined HLLTx was associated with prolonged waitlist times relative to bilateral sequential single-lung transplantation (median 556 vs 56 days, respectively), however waitlist mortality and/or delisting was comparable between groups.

Conclusions: Taken together, these findings highlight the opportunities and challenges facing combined (heart-) lung and liver transplantation in patients with multiorgan failure.

MeSH terms

  • Adult
  • Australia
  • Cystic Fibrosis* / surgery
  • End Stage Liver Disease*
  • Esophageal and Gastric Varices*
  • Gastrointestinal Hemorrhage
  • Humans
  • Liver Transplantation*
  • Lung
  • Lung Transplantation* / adverse effects
  • Retrospective Studies
  • Severity of Illness Index