Aim: to present an exceptional case of the combined pathology – an abnormal drainage of portal system to the coronary sinus, hypoplasia of the right branch of the portal vein and high pulmonary hypertension. Materials and methods. The patient P. of 22 years old arrived at congenital heart diseases surgical unit of V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs. After the examination he was diagnosed with congenital disease – abnormal confl uence of the portal vein in the right atrium. Insuffi ciency of the tricuspid valve 3–4. High pulmonary hypertension. Insuffi ciency of blood circulation of IIa, III functional class. After computer tomography there is no division of the portal vein. Vein is drained in the right atrium bypass with a diameter of 2,3 cm following from the portal vein to the coronary sinus. Results. Considering hopelessness of conservative therapy the only method of the radical help to the patient is two-stage surgical intervention – transplantation of a heart-pulmonary complex and liver transplantation. Conclusion. Presented rare clinical case demonstrates the combination of congenital anomalies of the portal system and the heart with high pulmonary hypertension, and is based on a functioning fetal venous duct (ductus venosus), bypassing the liver in the right atrium (coronary sinus) in combination with hypoplasia of the right portal vein.

Aim. To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fibrosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension. Description. Patient F., 15 years old, was diagnosed with Congenital Heart Disease - double-chambered right ventricle, ventricular septal defect. Fibromuscular subaortic stenosis. Aortic valve insufficiency II. Circulatory failure II A., functional class III. Extrahepatic portal hypertension. Splenomegaly. Thrombocytopenia. The state after the imposition of splenorenal anastomosis in September 1998 and the imposition of mesocaval H-shaped anastomosis in 1998. Non-functioning anastomoses. Patient indicated for surgical correction of CHD, however, given the low level of platelets, expressed splenomegaly, leucopenia, the patient was referred for a preliminary treatment to Rogachev's Pediatric Hematology, Oncology and Immunology Center. The treatment (stimulating thrombopoiesis using romiplostim) gave no significant effect. Platelet count reached 70-90 x 10(9). Seeing of frequent bleeding from esophageal varices, the patient underwent varix sclerosis. At the time of hospitalization - no esophageal varices. The patient appealed to V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs where he was recommended for surgical treatment. Result. The patient performed surgery: radical correction of CHD: resection of the stenosis of the outflow tract of the right ventricle, subaortic stenosis resection, closure of ventricular septal defect, AV plasty under cardiopulmonary bypass. The early postoperative period was uneventful. Leukopenia was observed to 1,2 x 10(9), thrombocytopenia 70-90 x 10(9). Despite the low level of platelets bleeding in pre-and postoperative period was not registered. Antibiotic therapy with tienam. Good postoperative results were obtained. Liver function was not affected. Conclusion. Presented clinical case demonstrates the possibility of surgical intervention of combined heart disease with cardiopulmonary bypass in patient with extrahepatic portal hypertension.