Pregnancy outcomes in women with Budd–Chiari syndrome or portal vein thrombosis – a multicentre retrospective cohort study

Abstract

Objective To evaluate current practice and outcomes of pregnancy in women previously diagnosed with Budd–Chiari syndrome and/or portal vein thrombosis, with and without concomitant portal hypertension. Design and setting Multicentre retrospective cohort study between 2008 and 2021. Population Women who conceived in the predefined period after the diagnosis of Budd–Chiari syndrome and/or portal vein thrombosis. Methods and main outcome measures We collected data on diagnosis and clinical features. The primary outcomes were maternal mortality and live birth rate. Secondary outcomes included maternal, neonatal and obstetric complications. Results Forty‐five women (12 Budd–Chiari syndrome, 33 portal vein thrombosis; 76 pregnancies) were included. Underlying prothrombotic disorders were present in 23 of the 45 women (51%). Thirty‐eight women (84%) received low‐molecular‐weight heparin during pregnancy. Of 45 first pregnancies, 11 (24%) ended in pregnancy loss and 34 (76%) resulted in live birth of which 27 were at term (79% of live births and 60% of pregnancies). No maternal deaths were observed; one woman developed pulmonary embolism during pregnancy and two women (4%) had variceal bleeding requiring intervention. Conclusions The high number of term live births (79%) and lower than expected risk of pregnancy‐related maternal and neonatal morbidity in our cohort suggest that Budd–Chiari syndrome and/or portal vein thrombosis should not be considered as an absolute contraindication for pregnancy. Individualised, nuanced counselling and a multidisciplinary pregnancy surveillance approach are essential in this patient population. Tweetable abstract Budd–Chiari syndrome and/or portal vein thrombosis should not be considered as an absolute contraindication for pregnancy.

Maternal and fetal outcomes in pregnant patients with non-cirrhotic portal hypertension: A systematic review and meta-analysis

“…A total of 12 studies were included in the final analysis. [16][17][18][19][20][21][22][23][24][25][26][27] Table 1 summarizes the characteristics of the included studies. Nine studies [16][17][18][19][20]22,23,25,26 were from single centers from various parts of India while three studies were multicentric studies from Europe.…”

“…Nine studies [16][17][18][19][20]22,23,25,26 were from single centers from various parts of India while three studies were multicentric studies from Europe. 21,24,27 Six studies [19][20][21][22]26,27 reported the outcomes in patients with only EHPVO, one study 24 reported the outcomes in only NCPH and five studies reported outcomes in combined patients. [16][17][18]23,25 Seven studies were of low quality, 16,18,19,[22][23][24]26 while five studies were of medium quality 17,20,21,25,27 (Supplemental Table 1).…”

“…21,24,27 Six studies [19][20][21][22]26,27 reported the outcomes in patients with only EHPVO, one study 24 reported the outcomes in only NCPH and five studies reported outcomes in combined patients. [16][17][18]23,25 Seven studies were of low quality, 16,18,19,[22][23][24]26 while five studies were of medium quality 17,20,21,25,27 (Supplemental Table 1).…”

“…The incidence of variceal bleeding in the intrapartum period was reported in all 12 studies with 510 pregnancies. [16][17][18][19][20][21][22][23][24][25][26][27] The pooled rate of variceal bleeding was 9.6% (95% CI: 4.8-14.4; I 2 = 77.4%, p = 0.000) (Figure 1). On subgroup analysis, there was no difference in the incidence of variceal bleeding between studies from India and Europe (p = 0.088).…”

“…The rate of spontaneous miscarriage was reported in all 12 studies with 510 pregnancies. [16][17][18][19][20][21][22][23][24][25][26][27] The pooled rate of spontaneous miscarriage in pregnant women NCPH was 11.9% (95% CI: 7.8-16.1; I 2 = 52.3%, p = 0.090) (Figure 2). On subgroup analysis, the pooled rate of spontaneous miscarriage was significantly higher in studies from Europe compared to India [21.1% (95% CI: 14.0-28.2) vs. 9.1% (95% CI: 5.4-12.9); p = 0.017].…”