Observational Study
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jul 28, 2017; 9(21): 945-952
Published online Jul 28, 2017. doi: 10.4254/wjh.v9.i21.945
Outcomes of pregnancy in patients with known Budd-Chiari syndrome
Faisal Khan, Ian Rowe, Bill Martin, Ellen Knox, Tracey Johnston, Charlie Elliot, Will Lester, Frederick Chen, Simon Olliff, Homoyon Mehrzad, Zergham Zia, Dhiraj Tripathi
Faisal Khan, Ian Rowe, Dhiraj Tripathi, Liver Unit, Queen Elizabeth Hospital, Birmingham B15 2GW, United Kingdom
Bill Martin, Ellen Knox, Tracey Johnston, Birmingham Women’s Hospital, Birmingham B15 2TG, United Kingdom
Charlie Elliot, Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield S10 2JF, United Kingdom
Will Lester, Frederick Chen, Haematology and Thrombophilia Centre, Queen Elizabeth Hospital, Birmingham B15 2GW, United Kingdom
Simon Olliff, Homoyon Mehrzad, Zergham Zia, Interventional Radiology, Queen Elizabeth Hospital, Birmingham B15 2GW, United Kingdom
Author contributions: Tripathi D conceived the study idea; Khan F and Tripathi D designed the study; Khan F conducted the analyses and drafted the manuscript; all authors contributed to discussing, editing and commenting on the manuscript.
Institutional review board statement: Study was approved the Audit office of the institution (attached).
Conflict-of-interest statement: There are no conflicts of interest to report.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Faisal Khan, Liver Unit, Queen Elizabeth Hospital, Mindelsohn Way, Birmingham B15 2TH, United Kingdom. faisalkhan@doctors.org.uk
Telephone: +44-121-6272000
Received: December 12, 2016
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
Abstract
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 pregnancy and the other in the post-partum period. There was no maternal mortality.

CONCLUSION

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.

Keywords: Budd-Chiari syndrome, Pregnancy, Portal hypertension, Pulmonary hypertension, Thrombophilia

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.