Hepatopulmonary syndrome and liver transplantation: a review of the peroperative management of seven paediatric cases.

Abstract

Until recently, hypoxaemia was considered as a relative contraindication for liver transplantation. The hepatopulmonary syndrome associated with a right to left shunt of blood through the lungs is reversible in adults and children after correction of the cirrhosis by liver transplantation. However, concerns have been raised regarding the risks of anaesthesia in such hypoxaemic patients. Since the peroperative management of children undergoing liver transplantation and suffering from hepatopulmonary syndrome and severe hypoxemia has never been described, we report here our experience in seven children. Despite the fact that severe arterial desaturation was recorded throughout the procedure, no major complications were recorded peroperatively. The postoperative intubation time was 58 +/- 21 h, five children being extubated while still hypoxaemic. All seven patients reversed their hepatopulmonary syndrome after a mean postoperative period of 24 +/- 10 weeks. This shows that liver transplantation can be successfully achieved in severely hypoxaemic children and that postoperative correction of the right to left shunt is then obtained.