Guideline sources The following summarized guidelines for the evaluation and management of portal vein thrombosis (PVT) are prepared by our editorial team based on guidelines from the American Society of Hematology (ASH 2023), the American Association for the Study of Liver Diseases (AASLD 2021), the American College of Gastroenterology (ACG 2020), the European Federation of Societies for Ultrasound (EFSU 2020), and the European Association for the Study of the Liver (EASL 2016). 1 2 3 4 5 Guidelines 1. Screening and diagnosis Clinical presentation: Assess for the possibility of acute PVT in any patient with abdominal pain. A Assess for the possibility of intestinal infarction in patients with severe and persistent abdominal pain, rectal bleeding, moderate or severe ascites, or multiorgan dysfunction. Monitor these patients closely for signs of deterioration. B 2. Classification and risk stratification https://web.pathway.md/diseases/rec0ru0bwEsT3D3Np 1/6 6/29/23, 11:20 PM Portal vein thrombosis Pathway Prognosis: insufficient evidence whether PVT in patients with cirrhosis is merely a reflection of progressive portal hypertension or independently causative of increased mortality, outside of liver transplant candidates. I Show 2 more 3. Diagnostic investigations Diagnostic imaging: As per AASLD 2021 guidelines, ensure a standardized documentation of initial site, extent, degree of luminal obstruction, and chronicity of clot formation in any patient with PVT, in order to make objective serial assessments of spontaneous regression or treatment response. E As per ACG 2020 guidelines: Obtain Doppler ultrasound as the initial noninvasive modality for diagnosis of PVT. B Obtain contrast-enhanced CT or MRI to assess the extension of thrombus into the mesenteric veins and to exclude tumor thrombus in patients with cirrhosis developing new portal and/or mesenteric vein thrombus. B As per EFSU 2020 guidelines, obtain contrast-enhanced ultrasound for differentiation between benign and malignant PVT. B As per EASL 2016 guidelines, obtain Doppler ultrasound as the first-line imaging test for acute PVT. Obtain CT for diagnostic confirmation and assessment of extension. A Show 2 more Evaluation for underlying causes: As per AASLD 2021 guidelines, exclude malignant venous obstruction attributable to HCC with appropriate contrast-enhanced imaging in patients with cirrhosis. E Show 2 more As per EASL 2016 guidelines, evaluate patients with PVT for underlying cirrhosis or obliterative portal venopathy. A Show 5 more 4. Medical management General principles: obtain immediate consultation with surgery, critical care, interventional radiology, and hematology in all patients with recent PVT and concern for intestinal ischemia. Initiate anticoagulation. Proceed to surgery in cases of intestinal infarction. E Show 2 more Indications for treatment: consider obtaining observation with serial imaging every 3 months without therapy in patients with cirrhosis with recent thrombosis of small intrahepatic sub- branches of the portal vein or minimally occlusive (< 50% obstruction of the lumen) thrombosis of the main portal vein. Consider initiating treatment later in case of progressive clot. E Show 2 more Indications for anticoagulation, non-cirrhotic PVT: https://web.pathway.md/diseases/rec0ru0bwEsT3D3Np 2/6 6/29/23, 11:20 PM Portal vein thrombosis Pathway As per AASLD 2021 guidelines, consider initiating directed antithrombotic therapy in patients without cirrhosis and with recent PVT, in order to avoid intestinal ischemia and prevent the development of chronic PVT with portal hypertension. E As per ACG 2020 guidelines, initiate anticoagulation in all patients with non-cirrhotic, acute symptomatic portal or mesenteric vein thrombosis in the absence of any contraindication. B Show 6 more As per EASL 2016 guidelines, initiate immediate anticoagulation therapy with LMWH in the absence of major contraindications to anticoagulation. A Show 2 more Indications for anticoagulation, PVT in cirrhosis: As per AASLD 2021 guidelines, consider initiating antithrombotic therapy in patients with cirrhosis with recent occlusive or partially occlusive (> 50% obstruction of the lumen) thrombosis of the main portal vein or mesenteric veins, in order to avoid thrombosis progression possibly hindering future liver transplantation or causing progression of portal hypertension. E As per ACG 2020 guidelines, initiate anticoagulation in patients with cirrhosis and: acute complete main PVT mesenteric vein thrombosis extension of PVT into mesenteric veins. B Show 6 more As per EASL 2016 guidelines, implement adequate prophylaxis for gastrointestinal bleeding before initiation of anticoagulation in patients with cirrhotic PVT. A Choice of anticoagulation: individualize the choice of agent for anticoagulant therapy (LMWH, vitamin K antagonists, and direct OACs) in patients with PVT. Consider consulting with a hematologist and/or expert hepatologist in deciding on anticoagulant agents and duration. E Show 2 more Duration of anticoagulation (testing for thrombophilia): Consider obtaining thrombophilia testing to guide anticoagulant treatment duration after primary treatment in patients with splanchnic venous thrombosis in a setting where anticoagulation would be discontinued. Consider continuing anticoagulation indefinitely in patients with thrombophilia. C Avoid obtaining thrombophilia testing to guide anticoagulant treatment duration after primary treatment in patients with splanchnic venous thrombosis in a setting where anticoagulation would be continued indefinitely. D Duration of anticoagulation, non-cirrhotic PVT: As per ACG 2020 guidelines, consider administering at least 6 months of anticoagulation in patients with non-cirrhotic, portal or mesenteric vein thrombosis without a demonstrable thrombophilia and when the etiology of the thrombosis is reversible. Offer indefinite anticoagulation in patients with non-cirrhotic, portal or mesenteric vein thrombosis and thrombophilia. C As per EASL 2016 guidelines: https://web.pathway.md/diseases/rec0ru0bwEsT3D3Np 3/6 6/29/23, 11:20 PM Portal vein thrombosis Pathway Complete at least 6 months of anticoagulation therapy in patients with non-cirrhotic, non- malignant PVT. A Continue anticoagulant therapy indefinitely in patients with PVT and an underlying myeloproliferative neoplasm. B Duration of anticoagulation, PVT in cirrhosis: As per ACG 2020 guidelines, consider administering anticoagulation for 6 months in patients with cirrhotic, acute portal or mesenteric vein thrombosis. Continue anticoagulation beyond this period in patients with cirrhotic, portal, or mesenteric vein thrombosis awaiting a liver transplant. C As per EASL 2016 guidelines, implement adequate prophylaxis for gastrointestinal bleeding before initiation of anticoagulation in patients with cirrhotic PVT. A Show 3 more Management of underlying causes: treat any underlying prothrombotic condition appropriately. B 5. Therapeutic procedures Endoscopic variceal ligation: consider performing endoscopic vatical ligation without stopping therapeutic anticoagulation. Initiate anticoagulation as soon as possible and not delayed until variceal eradication or adequate -blockade is achieved. E Portal vein recanalization: Consider performing portal vein recanalization followed by TIPS in liver transplant candidates with chronic PVT hindering a physiological anastomosis between the graft and recipient portal vein. Make this decision as part of a multidisciplinary management process, including surgical and interventional radiology expertise. E Consider performing portal vein recanalization followed by TIPS in patients with chronic PVT and recurrent bleeding and/or refractory ascites not manageable medically or endoscopically. E Transjugular intrahepatic portosystemic shunt: consider performing placement of TIPS in patients being liver transplant candidates with progressive PVT not responding to anticoagulation. C 6. Follow-up and surveillance Follow-up: Obtain CT to assess recanalization of the portal venous system at 6-12 months follow-up. B Screen for gastroesophageal varices in unrecanalized patients. A Evaluation of new thrombocytopenia: screen for heparin-induced thrombocytopenia in patients with a sudden unexplained platelet count fall 50% or to a value < 150 10 cells/L, especially if UFH is initiated. A https://web.pathway.md/diseases/rec0ru0bwEsT3D3Np 4/6 6/29/23, 11:20 PM Portal vein thrombosis Pathway Clinical findings Symptoms Past medical history Abdominal pain Gastroesophageal varices Chills Gastrointestinal bleeding Diarrhea HCC Fever Intestinal infarction Hematemesis Liver cirrhosis Hematochezia Malignancy Nausea Pancreatitis RUQ pain Vital signs Weight loss Hypotension Medication history Signs of shock Estrogens Integument exam Abdominal exam Jaundice Abdominal distension Hematological findings Ascites Splenomegaly Coagulopathy Polycythemia Lab findings WBC count serum ALT blood platelet count serum AST References 1. Douglas A Simonetto, Ashwani K Singal, Guadalupe Garcia-Tsao et al. ACG Clinical Guideline: Disorders of the Hepatic and Mesenteric Circulation. Am J Gastroenterol. 2020 Jan;115 1 18 40. Open 2. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 3. Christoph F Dietrich, Christian P llson Nols e, Richard G Barr et al. Guidelines and Good Clinical Practice Recommendations for Contrast-Enhanced Ultrasound CEUS in the Liver-Update 2020 WFUMB in Cooperation with EFSUMB, AFSUMB, AIUM, and FLAUS. Ultrasound Med Biol. 2020 Oct;46 10 2579 2604. Open 4. Patrick G Northup, Juan Carlos Garcia-Pagan, Guadalupe Garcia-Tsao et al. Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 366 413. Open 5. Saskia Middeldorp, Robby Nieuwlaat, Lisa Baumann Kreuziger et al. American Society of Hematology 2023 Guidelines for Management of Venous Thromboembolism: Thrombophilia Testing. Blood Adv. 2023 May 17;bloodadvances.2023010177. Open https://web.pathway.md/diseases/rec0ru0bwEsT3D3Np 5/6 6/29/23, 11:20 PM Portal vein thrombosis Pathway 6. Campbell Tait, Trevor Baglin, Henry Watson et al. Guidelines on the investigation and management of venous thrombosis at unusual sites. Br J Haematol. 2012 Oct;159 1 28 38. Open https://web.pathway.md/diseases/rec0ru0bwEsT3D3Np 6/6