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Outcomes of pregnancy in patients with known Budd-Chiari syndrome

World Journal of Hepatology 2017年第21期9卷 945-952页

作者： Faisal Khan Ian Rowe Bill Martin Ellen Knox Tracey Johnston Charlie Elliot Will Lester Frederick Chen Simon Olliff homoyon mehrzad Zergham Zia Dhiraj Tripathi Liver Unit Queen Elizabeth Hospital Birmingham Women’s Hospital Pulmonary Vascular Disease Unit Royal Hallamshire Hospital Haematology and Thrombophilia Centre Queen Elizabeth Hospital Interventional Radiology Queen Elizabeth Hospital

AIM To analyse the risk of pregnancy(a prothrombotic state) in patients with Budd-Chiari Syndrome(BCS). METHODS Retrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015. RESULTS 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 pregnancyand the other in the post-partum period. There was no maternal *** 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.

关键词： Budd-Chiari syndrome Pregnancy Portal hypertension Pulmonary hypertension Thrombophilia

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