Volume 29, Issue 1 e70036
CASE REPORT

Decisions Using Intra-Operative Gross Assessment of Fontan-Associated Liver Disease: Implications for Heart-Only Transplantation

Diana Milagros Torpoco Rivera

Corresponding Author

Diana Milagros Torpoco Rivera

Division of Cardiology, the Children's Hospital of Michigan, Department of Pediatrics, Central Michigan University College of Medicine, Detroit, Michigan, USA

Correspondence:

Diana Milagros Torpoco Rivera ([email protected])

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Ke-You Zhang

Ke-You Zhang

Division of Gastroenterology, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Noelle Ebel

Noelle Ebel

Division of Gastroenterology, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Rachel Bensen

Rachel Bensen

Division of Gastroenterology, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Elisabeth Martin

Elisabeth Martin

Department of Cardiothoracic Surgery, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Michael Ma

Michael Ma

Department of Cardiothoracic Surgery, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Carlos O. Esquivel

Carlos O. Esquivel

Department of Surgery, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Manchula Navaratnam

Manchula Navaratnam

Department of Anesthesia, Division of Pediatric Cardiology at Lucile Packard Children's Hospital at Stanford University, Palo Alto, California, USA

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Seth A. Hollander

Seth A. Hollander

Department of Pediatrics (Cardiology), Stanford University, Stanford, California, USA

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David N. Rosenthal

David N. Rosenthal

Department of Pediatrics (Cardiology), Stanford University, Stanford, California, USA

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Sharon Chen

Sharon Chen

Department of Pediatrics (Cardiology), Stanford University, Stanford, California, USA

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First published: 27 January 2025

Funding: The authors received no specific funding for this work.

ABSTRACT

Introduction

Intraoperative gross liver inspection at the time of transplant can be used in determination of which failing Fontan patients should undergo heart-only transplantation (HT) versus combined heart–liver transplantation (CHLT). We describe the outcomes of Fontan patients listed for CHLT who underwent HT based on the gross pathology of the liver at time of transplant.

Case Report

Six Fontan patients, ages 15 (IQR12,16) years, listed for CHLT received HT because of reassuring gross findings of the liver at the time of transplant. All patients survived to hospital discharge, after median hospital length of stay of 59 (IQR 42–77) days. Vasoplegia, prolonged need for milrinone because of diastolic dysfunction, acute kidney injury, and infection were common complications. Five patients had acute cellular rejection within 35 days of heart transplant. At a median of 17 (IQR 10, 19) months from HT, all continue to have abnormal findings by abdominal imaging, with development of new hepatic nodules in three patients.

Summary

Intraoperative gross liver inspection at the time of transplant can enable successful HT in Fontan patients with liver disease. However, a prolonged recovery period should be anticipated, with serial post-HT liver assessments and vigilant monitoring for rejection. The long-term liver prognosis remains uncertain.

Conflicts of Interest

The authors declare no conflicts of interest.

Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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