Fate of the liver in the survivors of adult heart transplant for a failing Fontan circulation

Christopher R Broda; Rafael Alonso-Gonzalez; Anand Ghanekar; Aliya Gulamhusein; Michael McDonald; Adriana Luk; Jeremy Kobulnik; Filio Billia; Jane Heggie; Marjan Jariani; Osami Honjo; David Barron; Edward Hickey; Susan L Roche

doi:10.1016/j.healun.2021.10.020

Fate of the liver in the survivors of adult heart transplant for a failing Fontan circulation

J Heart Lung Transplant. 2022 Mar;41(3):283-286. doi: 10.1016/j.healun.2021.10.020. Epub 2021 Nov 11.

Authors

Affiliations

¹ Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Pediatric and Adult Congenital Heart Disease, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas.
² Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
³ Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
⁴ Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada; Toronto Centre for Liver Disease, Francis Family Liver Clinic, University Health Network, Toronto, Ontario, Canada.
⁵ Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Department of Anesthesiology and Pain Medicine, University of Toronto, Toronto, Ontario, Canada.
⁶ Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
⁷ Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Pediatric and Adult Congenital Heart Disease, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas.
⁸ Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada. Electronic address: [email protected].

PMID: 34953719
DOI: 10.1016/j.healun.2021.10.020

Abstract

The Fontan circulation has inherent long-term vulnerabilities such that adult Fontan patients now comprise the largest, most rapidly growing subgroup of adult congenital heart disease referred for transplant assessment. Almost all have Fontan Associated Liver Disease (FALD). There is an absence of mid to late hepatic outcome data after heart transplant alone. Therefore, we analyzed outcomes of survivors of heart only transplant in patients with failing Fontan circulation. Including all 10 of our adult Fontan patients surviving >1 year after isolated heart transplant, we report evolution of their clinical features, bloodwork, hemodynamic data, and liver ultrasound findings over a median of 4.7 years. Nonprogression of FALD, resolution of ascites and freedom from hepatocellular carcinoma in the mid-term highlight the outcomes in this selected group once normal cardiac output and venous pressures are established by heart transplant.

Keywords: Fontan associated liver disease; Fontan failure; adult congenital heart disease; heart transplantation.

MeSH terms

Adult
Female
Fontan Procedure*
Heart Defects, Congenital / surgery*
Heart Transplantation*
Humans
Liver Diseases / epidemiology*
Male
Middle Aged
Postoperative Complications / epidemiology*
Retrospective Studies
Treatment Failure
Treatment Outcome