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Introduction: As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation. Methods: We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006–2022. Results: The 30‐day, 1‐year, 5‐year and 10‐year survival rate was 90%, 80%, 73% and 73% respectively. Sixty percent of patients met a composite comorbidity of needing either post‐transplant mechanical circulatory support, renal replacement therapy or tracheostomy. Cardiopulmonary bypass time > 283 min (4.7 h) and meeting the composite comorbidity were associated with mortality by Kaplan Meier analysis. Conclusion: Further study to mitigate early mortality and the above comorbidities as well as the high risk of bleeding and vasoplegia in this patient population is warranted. [ABSTRACT FROM AUTHOR]

The accuracy of liver biopsy to stage fibrosis due to Fontan‐associated liver disease (FALD) remains unclear. We compared the results of biopsy pre–combined heart and liver transplantation (CHLT) to the results of whole liver explant. Liver biopsy and explants from 15 Fontan patients (ages 16‐49, median 28 years) were retrospectively reviewed. Staging was as follows: stage 0: no fibrosis, stage 1: pericellular fibrosis, stage 2: bridging fibrosis, and stage 3: regenerative nodules. There is no stage 4. Clinical characteristics including Model of End‐stage Liver Disease eXcluding INR and Varices, Ascites, Splenomegaly, and Thrombocytopenia (VAST) scores were collected, and descriptive statistics and Mann‐Whitney U tests were used to analyze the data. All patients had biopsies with at least bridging fibrosis, and all had nodularity on explant; transjugular biopsy never overestimated fibrosis. Explant showed higher‐grade fibrosis (stage 3) than pre‐CHLT biopsy (stage 2) in 6 of 15 patients and equal grade of fibrosis (stage 3) in 9 of 15 patients. Though clinical characteristics varied significantly, VAST score was ≥2 in all but two patients. Transjugular liver biopsy does not overestimate and can underestimate fibrosis in Fontan patients undergoing CHLT, likely due to the patchy nature of fibrosis in FALD. [ABSTRACT FROM AUTHOR]

Patients with failing Fontan physiology and liver cirrhosis are being considered for combined heart and liver transplantation. We performed a retrospective review of our experience with en bloc combined heart and liver transplantation in Fontan patients > 10 years old from 2006 to 18 per Institutional Review Board approval. Six females and 3 males (median age 20.7, range 14.2‐41.3 years) underwent en bloc combined heart and liver transplantation. Indications for heart transplant included ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities. Indication for liver transplant included portal hypertension and cirrhosis. Median Fontan/single ventricular end‐diastolic pressure was 18/12 mm Hg, respectively. Median Model for End‐Stage Liver Disease excluding International Normalized Ratio score was 10 (7‐26), eight patients had a varices, ascites, splenomegaly, thrombocytopenia score of ≥ 2, and all patients had cirrhosis. Median cardiopulmonary bypass and donor ischemic times were 262 (178‐307) and 287 (227‐396) minutes, respectively. Median intensive care and hospital stay were 19 (5‐96) and 29 (13‐197) days, respectively. Survival was 100%, and rejection was 0% at 30 days and 1 year post‐transplant. En bloc combined heart and liver transplantation is an acceptable treatment in the failing Fontan patient with liver cirrhosis. [ABSTRACT FROM AUTHOR]