Published online Jul 28, 2017. doi: 10.4254/wjh.v9.i21.945
Peer-review started: December 13, 2016
First decision: March 13, 2017
Revised: April 14, 2017
Accepted: June 30, 2017
Article in press: July 3, 2017
Published online: July 28, 2017
To analyse the risk of pregnancy (a prothrombotic state) in patients with Budd-Chiari Syndrome (BCS).
Retrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015.
Out of 53 females with BCS, 7 women had 16 pregnancies. Median age at diagnosis of BCS in these women was 25 years (range 21-34 years). At least one causal factor for BCS was identified in 6 women (86%). Six women had undergone radiological decompressive treatment. All patients had anticoagulation. Six fetuses were lost before 20 wk gestation in 2 women. There were 9 deliveries over 32 wk gestation and one delivery at 27 wk. All infants did well. Seven babies were born by emergency caesarean section. There were no cases of thrombosis. Two patients had notable vaginal (PV) bleeding in 3 pregnancies. None of the patients had variceal haemorrhage. Two patients were diagnosed with pulmonary hypertension, one during pregnancy and the other in the post-partum period. There was no maternal mortality.
Maternal outcomes in patients with treated BCS are favourable and fetal outcomes beyond 20 wk gestation are good. There has been increased rate of caesarean section. Pulmonary hypertension is an important finding that needs further validation. These patients should be managed in centers experienced in treating high-risk pregnancies.
Core tip: Pregnancy is a prothrombotic state and can cause adverse outcome in patients with Budd-Chiari syndrome (BCS). In our study, maternal outcome in patients with known and treated BCS was good. However, most deliveries were carried out by emergency caesarean section (7/10). There was high incidence of placental disease leading to caesarean section. Fetal outcome beyond 20 wk gestation was also good. With careful monitoring of anti-coagulation, there were no cases of thrombosis and only a minority of patients had noteworthy bleeding complications. Development of pulmonary hypertension in two patients several years after TIPSS is an important finding that warrants further studies.