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Published on: September 13, 2014
Decisions Using Intra-Operative Gross Assessment of Fontan-Associated Liver Disease: Implications for Heart-Only
Diana Milagros Torpoco Rivera1, Ke-You Zhang2, Noelle Ebel2
1Division of Cardiology, the Children's Hospital of Michigan, Department of Pediatrics, Central Michigan University College of Medicine, Detroit, Michigan, USA.
Insights
Intraoperative liver inspection allowed heart-only transplantation (HT) in Fontan patients, but prolonged recovery and uncertain long-term liver outcomes necessitate vigilant monitoring for complications and rejection.
Area of Science:
- Cardiology
- Hepatology
- Transplantation Surgery
Background:
- Fontan patients with liver disease face decisions between heart-only transplantation (HT) and combined heart-liver transplantation (CHLT).
- Intraoperative gross liver inspection can guide this decision-making process.
Observation:
- Six Fontan patients listed for CHLT received HT based on reassuring intraoperative liver findings.
- All patients survived to discharge with a median hospital stay of 59 days.
- Common complications included vasoplegia, prolonged milrinone use, acute kidney injury, and infection.
Findings:
- Five patients experienced acute cellular rejection within 35 days post-HT.
- All patients showed persistent abnormal abdominal imaging findings at a median of 17 months post-HT.
- Three patients developed new hepatic nodules.
Implications:
- Intraoperative liver assessment can facilitate successful HT in Fontan patients with liver disease.
- Anticipate prolonged recovery and the need for serial liver assessments and rejection monitoring.
- Long-term liver prognosis in these patients remains uncertain.
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.
