In the absence of cirrhosis, recent portal vein thrombosis (PVT) rarely resolves spontaneously. Therefore, at diagnosis, anticoagulation should be started immediately at a therapeutic dosage.
Because of the increased risk of heparin-induced thrombocytopenia, the use of unfractionated heparin is not generally recommended and may only be reserved for special situations (e.g. glomerular filtration rate <30 ml/min, pending invasive procedures).
As a primary treatment option for recent PVT in the absence of cirrhosis, start with low-molecular-weight heparin (LMWH) and switch to vitamin K antagonists when possible. DOACs can be considered the primary option in selected cases in the absence of so-called “triple positive” anti-phospholipid syndrome, although data are limited.
Anticoagulation should be given for at least 6 months in all patients with recent PVT in the absence of cirrhosis.
After 6 months, long-term anticoagulation is recommended in patients with a permanent underlying prothrombotic state and should also be considered in patients without an underlying prothrombotic state.
If anticoagulation is discontinued, D-dimers <500 ng/ml 1 month after discontinuation may be used to predict a low risk of recurrence.
In patients without cirrhosis who do not develop complications of recent PVT despite the absence of portal vein recanalisation, interventions other than anticoagulation are not required.
A follow-up contrast-enhanced CT scan should be performed 6 months after recent PVT.
Because of the risk of recurrence of splanchnic vein thrombosis, patients need to be followed up, irrespective of the discontinuation of anticoagulation.
The risk of intestinal infarction and organ failure is increased in patients with recent PVT and (i) persistent severe abdominal pain despite anticoagulation therapy, (ii) bloody diarrhoea, (iii) lactic acidosis, (iv) bowel loop distention, or (v) occlusion of second order radicles of the superior mesenteric vein. Therefore, a multidisciplinary approach with early image-guided intervention, thrombolysis and surgical intervention should be considered in referral centres.
In patients with past PVT or cavernoma, including those with incomplete resolution of recent PVT at 6 months, long-term anticoagulation is recommended in patients with a permanent underlying prothrombotic state and should also be considered in patients without an underlying prothrombotic state.
No data are available to recommend or discourage anticoagulation in childhood-onset past PVT or cavernoma in the absence of an underlying prothrombotic state.
In patients with past PVT or cavernoma not yet receiving anticoagulants, anticoagulation should be started after adequate prophylaxis for portal hypertensive bleeding has been initiated in patients with high-risk varices.
Mesenteric-left portal vein bypass (Meso-Rex operation) should be considered in all children with complications of portal cavernoma, and these patients should be referred to centres with experience in treating this condition.
Patients with refractory complications of PVT or cavernoma should be referred to expert centres to consider percutaneous recanalisation of the portal vein or other vascular interventional procedures.
Corrigendum to ‘Baveno VII – Renewing consensus in portal hypertension’ [J Hepatol (2022) 959-974] Journal of Hepatology, Vol. 77, Issue 2