Title: Experience and results of liver transplantation in patients with alagille syndrome at our center
Source: Experimental and Clinical Transplantation 2024, 22 (Suppl 5): 94-99
Date of publication: October 2024
Publication type: Article
Abstract: Objectives: Alagille syndrome is an autosomal recessive disorder with multisystemic involvement. Patients with Alagille syndrome have many accompanying anomalies such as atypical facial appearance, posterior embryotoxon, butterfly vertebra, cardiac anomalies, and biliary problems. Liver and heart complications can determine the mortality and morbidity of patients with Alagille syndrome. The scarcity of intrahepatic bile ducts leads to cholestatic liver damage. In the heart, although the disease most commonly causes peripheral pulmonary stenosis, patients can present with various structural heart diseases. These cardiac anomalies make intraoperative and postoperative management difficult in patients who need liver transplant. Here, we present 10 cases of liver transplant due to Alagille syndrome.
Materials and methods: From 1988 to the present, our center performed 724 liver transplants, with 366 being pediatric cases; among these, 10 were due to Alagille syndrome. We retrospectively examined patient morphological findings, cardiac pathology, and perioperative complications.
Results: Average weight and age of patients were 11 kg and 4.9 years, respectively. Eight patients had atypical facial appearance, 5 had posterior embryotoxon, and 6 had butterfly vertebra. Portoenterostomy (Kasai procedure) was performed in 3 patients because of suspicion of early biliary atresia. Cardiac pathology was present in 8 patients, with peripheral pulmonary stenosis being the most common (present in 8 patients). One patient had cardiac catheterization, with right ventricular and pulmonary artery pressures measured at 110 and 37 mm Hg, respectively. Two patients required preoperative pulmonary angioplasty. Two patients had double superior vena cava. No complications occurred in any patient during the anhepatic phase with appropriate volume replacement. No patients had early mortality.
Conclusions: Cardiac anomalies should be identified in patients with Alagille syndrome to minimize complications before liver transplant. If necessary, cardiac catheterization should be performed for pulmonary stenosis. Peripheral pulmonary stenosis does not constitute an absolute contraindication to liver transplant.
