Results of our corrective procedure in 71 cases of Budd-Chiari Syndrome

Abstract

Budd-Chiari syndrome is defined as hepatic venous outflow obstruction or stenosis, resulting in portal hypertension from congestive liver failure. We present our original surgical procedures and results in 71 consecutive Budd-Chiari syndrome patients treated at our institution. Operative procedures: The patients were placed in the left lateral position under unilateral ventilation and then the lateral site of the hepatic inferior vena cava and the right atrium were exposed by dividing the 1/3 lateral site of diaphragm circularly via right 6th intercostal thoracotomy and upper median laparotomy. After partial cardiopulmonary bypass via the right femoral artery and venous cannulation, the hepatic inferior vena cava was clamped and incised longitudinally. Our procedure involves autologous pericardium patch augmentation of the stenosed or obstructed hepatic inferior vena cava and re-open as many occluded hepatic veins as possible by a resecting venous wall including liver parenchyma, resulting in normal hepatic circulation. There are two (2.8%) perioperative death from liver failure and arrhythmia. During mean follow up period of 9.7 (0.04-29.8) years, 1-, 5- and 10-year mortality were 95.6%, 88.3% and 71.7%, respectively. Our corrective procedures for Budd-Chiari syndrome might have long-term survival and recovery in hepatic functions.