ДВУХЭТАПНОЕ ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ РЕБЕНКА ОДНОГО ГОДА С ВРОЖДЕННЫМ ПОРОКОМ СЕРДЦА И БИЛИАРНЫМ ЦИРРОЗОМ

Aim: Clinical case of successful two-stage surgical treatment of a 1-year-old child with congenital heart disease and biliary cirrhosis is represented in this article. At the fi rst day of life laparotomy was performed because of high intestinal obstruction. Kasai procedure and Roux-en-Y choledochojejunostomy were per- formed on 12 th day and at the end of second month of life, respectively. Liver biopsy showed the signs of biliary cirrhosis. At the same time ventricular septal defect and atrial septal defect with pulmonary hyper- tension were diagnosed. The fi rst step of treatment was the surgical septal defects closure. No complications during procedure, cardiopulmonary bypass and post-operative period were registered. There were no nega- tive effects on liver function after cardiac surgery. 11 months later living-donor liver transplantation was performed without any complications. Patient was discharged at 35 th post-transplant day with stable graft function.