Once the diagnosis of porto-sinusoidal vascular disorder (PSVD) is made, patients should be screened for associated immunological diseases, prothrombotic or genetic disorders and exposure to drugs/toxins.
Endoscopic screening for gastro-oesophageal varices is required at diagnosis of PSVD.
The non-invasive Baveno VII criteria for screening of oesophageal varices used in patients with cirrhosis cannot be applied to patients with PSVD.
During follow-up, the frequency of endoscopic screening for varices has not yet been defined. Management according to cirrhosis guidelines is recommended, except for stopping rules.
There is insufficient data on which therapy should be preferred for portal hypertension prophylaxis in PSVD. Management according to cirrhosis guidelines is recommended.
A contrast-enhanced CT scan is suggested at diagnosis of PSVD in order to assess the anatomy/patency of the portal venous system and potential portosystemic collaterals.
Screening for PVT in patients with PSVD: there is no data on the best screening method and interval. Doppler ultrasound every 6 months is suggested in patients with PSVD and features of portal hypertension. In case of abdominal pain, Doppler ultrasound or cross-sectional imaging should be performed to rule out splanchnic vein thrombosis.
No recommendation can be made regarding anticoagulation therapy to prevent the development of PVT in PSVD.
In those patients developing PVT, anticoagulant therapy should be started according to recommendations for non-cirrhotic PVT.
TIPS can be considered to treat severe complications of portal hypertension. Underlying/associated conditions, which negatively impact post-TIPS outcome, must be taken into account in making individual decisions regarding TIPS insertion.
Liver transplantation is an option in selected patients with PSVD and severe or refractory complications of portal hypertension or with advanced liver dysfunction. Indications should be discussed in expert centres.
Corrigendum to ‘Baveno VII – Renewing consensus in portal hypertension’ [J Hepatol (2022) 959-974] Journal of Hepatology, Vol. 77, Issue 2