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

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.