Pregnancy After In Vitro Fertilization in Budd–Chiari Syndrome Secondary to Polycythemia Vera: Multidisciplinary Management and Perioperative Considerations

Abstract

Budd–Chiari syndrome (BCS) is a rare hepatic vascular disorder frequently associated with prothrombotic conditions such as myeloproliferative neoplasms. Pregnancy and assisted reproductive technologies further increase thrombotic and portal-hypertensive risk. We report a successful in vitro fertilization–conceived pregnancy in a 35-year-old woman with chronic BCS secondary to JAK2-positive polycythemia vera. The pregnancy was complicated by ovarian hyperstimulation syndrome and severe intrahepatic cholestasis of pregnancy. Progressive portal hypertension with worsening esophageal varices prompted planned preterm delivery at 34 weeks’ gestation. A multidisciplinary team performed cesarean delivery under epidural anesthesia with prophylactic perioperative terlipressin administration and thromboprophylaxis. Maternal and neonatal outcomes were favorable. With careful multidisciplinary management, pregnancy in women with stable BCS is feasible.