Budd–Chiari Syndrome and Pregnancy—A Review

Abstract

Pregnancy is a hypercoagulable state, increasing the risk of venous thrombosis, including Budd–Chiari syndrome (BCS). Historically, pregnancy was contraindicated in BCS due to risks like hepatic dysfunction, thrombosis, bleeding, and poor fetal outcomes. However, better diagnostic modalities, greater awareness, and treatment advances, such as anticoagulant therapy, endovascular interventions like hepatic vein angioplasty with or without stenting, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation (LT), have enabled more favorable outcomes. When BCS presents during pregnancy, diagnosis can be challenging, often mimicking other pregnancy-related liver conditions. Doppler ultrasonography is the preferred diagnostic tool during pregnancy, with cross-sectional imaging reserved for doubtful cases and planning intervention. Anticoagulation is the cornerstone of medical therapy for BCS diagnosed in pregnancy, preventing thrombus progression. Radiological interventions like hepatic vein stenting and TIPS are options, particularly for those not responding to medical therapy, though radiation exposure is a consideration, and dose-reduction strategies are employed. LT is a consideration for acute liver failure, with good maternal but suboptimal fetal outcomes. For women with pre-existing BCS planning pregnancy, preconceptional management is crucial. This includes individualized risk assessment, optimizing BCS treatment, and screening for thrombophilia. Delayed diagnosis, advanced age, and progression to cirrhosis may all contribute to infertility in BCS, which need to be considered. However, successful BCS treatment can improve fertility and pregnancy outcomes. Antenatal, perinatal, and postpartum management requires careful monitoring of liver function, portal hypertension, anticoagulation, and fetal well-being, aimed at preventing complications like hemorrhage. Proactive management significantly improves the prognosis for pregnancy in BCS patients.