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\begin{document}

\title{Mini commentary on BJOG-21-0823: Pregnancy outcomes in women with
Budd-Chiari syndrome or portal vein thrombosis - A multicentre
retrospective cohort study.}



\author[1]{Yooyun Chung}%
\author[2]{Michael Heneghan}%
\affil[1]{King's College London}%
\affil[2]{King's College Hospital}%


\vspace{-1em}



  
  \date{June 08, 2024}


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\textbf{Mini commentary on BJOG-21-0823: Pregnancy outcomes in women
with Budd-Chiari syndrome or portal vein thrombosis - A multicentre
retrospective cohort study.}

Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT) are rare
thrombotic disorders which can affect females of reproductive age.
Physiological changes in pregnancy may result in or exacerbate
pre-existing known portal hypertension related issues associated with
these conditions. These conditions may also present de-novo in pregnancy
with acute onset ascites or variceal haemorrhage. Both in pregnancy and
in the non-pregnant state, in those with established disease, in
general, the overall balance of risk, favours continued anticoagulation.

The study by Wiegers et al. has shown favourable maternal and foetal
outcomes once greater than 20 weeks gestation is reached in patients
with BCS and/or PVT. However, the risk of preterm birth and early
pregnancy loss remains. These results are in keeping with the recent
study by Andrade et al. (Journal of Hepatology 2018; 69: 1242-1249)
looking at pregnancy outcomes from 24 pregnancies in 16 women with
idiopathic non-cirrhotic portal hypertension (INCPH). Rautou et al.
(Journal of Hepatology 2009; 51: 47-54) reviewed 24 pregnancies in 16
women with BCS and also reported similar findings. Taken as a whole,
these results support the concept that patients with vascular liver
disease can achieve favourable pregnancy outcomes but warrant careful
consideration in pregnancy.

Preconception counselling is a crucial opportunity to optimise patients
with vascular liver disease who are considering pregnancy. This can be
achieved in a multidisciplinary forum with input from obstetricians,
haematologists and hepatologists. It is useful to identify those women
with significant portal hypertension and varices before pregnancy so
that appropriate surveillance and eradication with endoscopic band
ligation and/or prophylaxis with beta blockers is undertaken. If
pregnancy is achieved before surveillance, then a second trimester
endoscopy for those with significant portal hypertension should be
performed. In patients with portal and mesenteric vein thrombosis,
magnetic resonance imaging of the pelvis may be needed to assess for the
presence of abdominal wall/pelvic varices. This stratifies the risk of a
variceal bleed and allows planning of the mode of delivery (caesarean,
vaginal or assisted vaginal delivery). Wiegers et al. reported 2
variceal bleeds in pregnancy but did not find a significant association
with adverse maternal outcomes, though this may be related to the low
number of patients. Andrade et al. also reported 2 variceal bleeds
including 1 patient with PVT without adequate endoscopic prophylaxis who
required a portosystemic shunt. This outlines the need for appropriate
screening and portal hypertension management according to findings.

The majority of patients with BCS and PVT have an underlying
pro-thrombotic tendency and the intra-partum and the post-partum periods
are associated with thrombotic events. Vitamin K antagonists are
historically the commonest anticoagulation used in BCS and PVT which
should be switched to low molecular heparin ideally before conception.
The use of anticoagulation is more common in patients with BCS and PVT
than in INCPH (38/45 women in BCS and/or PVT compared to 4/16 in INCPH).
4 out of 6 women with BCS and/or PVT who experienced post-partum
haemorrhage (PPH) were on anticoagulation. 2 patients with INCPH had PPH
whilst on anticoagulation which may be confounded by the
thrombocytopenia and type-2 error. In the study by Rautou et al., 17/24
pregnancies received anticoagulation and the 4 women who experienced
post-partum bleeding (vaginal or intrauterine/parietal haematoma) were
on anticoagulation which includes one woman with an ectopic pregnancy.
No maternal deaths were reported in the three studies and the continued
use of anticoagulation when indicated is safe and appropriate.

The mode of delivery did not affect the risk of PPH in the three
studies. The mode of delivery should be decided based on the individual
risk profile taking into account the severity of portal hypertension,
distribution of venous thrombosis, presence of coagulopathy and
thrombocytopenia, obstetric indications and the presence of oesophageal
or abdominal wall/pelvic varices.

The live birth rates may be lower in patients with BCS compared to PVT
(75\% versus 82\% after excluding first trimester pregnancy loss) but
due to the low number of patients it remains difficult to interpret the
results in this study. In the study by Andrade et al., all 18
pregnancies reaching 20 weeks gestation were delivered with 2 infant
deaths (both preterm births). Rautou et al. reported 16/17 live births
in pregnancies reaching 20 weeks gestation.

To conclude, patients with BCS and PVT after 20 weeks gestation and
appropriate planning can have a reasonable expectation for delivery and
successful outcomes. Preconception counselling and antenatal care with
multidisciplinary input is key to achieving this goal.

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