Pregnancy in Budd-Chiari Syndrome

Budd-Chiari syndrome (BCS) frequently affects women of childbearing age. Pregnancy is a prothrombotic state and can trigger BCS in women with an underlying prothrombotic condition. Therefore, such women should also be screened for other prothrombotic disorders. Earlier studies reported that women with BCS could be at risk of developing severe exacerbation of their underlying disease during pregnancy. Recent studies showed that good maternal outcome could be achieved with current treatment modalities and close surveillance of BCS during pregnancy. The reported maternal outcomes in patients with treated and stabilized BCS are favourable, and foetal outcomes beyond 20 weeks gestation are good. Increased rate of caesarean section and preterm deliveries have been reported though. In BCS patients wishing to become pregnant, should be screened for the presence of esophageal varices and appropriate prophylaxis of variceal haemorrhage should be implemented. Large or 'at-risk' varices should be eradicated with endoscopic band ligation. Once pregnant, gastroscopy should again be performed in second trimester, regardless of previous prophylaxis, as risk of variceal bleeding in patients with portal hypertension is the highest during the second trimester. Management of anticoagulation and delivery are best undertaken by a multi-disciplinary team experienced in dealing with high-risk pregnancies. Assisted vaginal delivery with adequate analgesia is preferable mode of delivery and caesarean section reserved for obstetric indications. BCS cannot be considered contraindicated to pregnancy in stable patients.