Outcomes of liver transplantations in patients with alagille syndrome with high pulmonary arterial pressure performed in our center
Adem Safak1, Emre Karakaya1, Figen Ozcay2, Birgul Varan3, Sedat Yildirim1, Mehmet A. Haberal1.
1Department of General Surgery, Division of Transplantation, Baskent University, Ankara, Turkey; 2Department of Pediatric Gastroenterology, Baskent University, Ankara, Turkey; 3Department of Pediatric Cardiology, Baskent University, Ankara, Turkey
Introduction: Alagille syndrome (AGS) is an autosomal recessive disorder with multisystemic involvement. The organs determining the mortality and morbidity of patients with Alagille syndrome are the liver and heart. The scarcity of intrahepatic bile ducts leads to cholestatic liver damage. In the heart, although it most commonly causes peripheral pulmonary stenosis, it can present with various structural heart diseases. In this study, we present 10 cases of liver transplantation due to Alagille syndrome.
Methods: From 1988 to the present, our center performed 724 liver transplants, with 366 being pediatric cases. Liver transplantation was performed on 10 pediatric patients due to Alagille syndrome. We retrospectively examined these patients in terms of morphological findings, cardiac pathology, and perioperative complications.
Results: The average weight of the patients was 11 kg, and the average age was 4.9 years. Atypical facial appearance was present in 8 patients. Posterior embryotoxon was observed in 5 patients, and butterfly vertebra was present in 6 patients. Kasai surgery was performed in 3 patients due to suspicion of early biliary atresia. Cardiac pathology was present in 8 patients, with peripheral pulmonary stenosis being the most common (present in 8 patients). Cardiac catheterization was performed on a patient with the highest right ventricular pressure measured at 110 mmHg. Pulmonary artery pressure was found to be 37 mmHg. Preoperative pulmonary angioplasty was performed in 2 patients. Two patients had double superior vena cava. No complications occurred in any patient during the anhepatic phase with appropriate volume replacement. There was no early mortality in any of the patients.
Conclusion: Patients with Alagille syndrome should be well prepared cardiac-wise to minimize complications before liver transplantation. Anomalies should be identified, and if necessary, cardiac catheterization should be performed for pulmonary stenosis. Peripheral pulmonary stenosis does not constitute an absolute contraindication in liver transplant candidates.
