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Portal and Hepatic Venous Thrombosis
Jennifer R. Buckley, MD; Brandt C. Wible, MD
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KEY FACTS

  • Terminology

    • Preprocedure

      • Procedure

        • Post Procedure

          TERMINOLOGY

          • Definitions

            • Hepatic venous outflow obstruction
              • 3 categories based on level of obstruction
                • Venoocclusive disease: Sinusoids and terminal venules
                  • Toxin-induced nonthrombotic obstruction; often occurs after stem cell transplant, chemotherapy, or radiotherapy
                • Budd-Chiari syndrome (BCS)
                  • Pain, ascites, liver enlargement caused by hepatic vein occlusion
                • Congestive hepatopathy
                  • Passive hepatic congestion, usually related to right heart failure (e.g., tricuspid regurgitation, cor pulmonale)
              • Associated problems of hepatic venous outflow obstruction
                • Cirrhosis, portal hypertension (HTN), hepatic/portal vein thrombosis
            • Hepatic vein thrombosis (HVT): Acute or chronic, complete or partial occlusion of hepatic veins ± extension into inferior vena cava (IVC)
              • Usually due to BCS
                • Primary
                  • Intraluminal thrombosis or webs
                  • Atypical myeloproliferative disease present in 50%
                  • Etiologies also include pregnancy, polycythemia vera, Behçet disease, paroxysmal nocturnal hemoglobinuria
                • Secondary
                  • Nonthrombotic; intraluminal invasion (e.g., tumor) or extraluminal compression
              • Can lead to portal HTN and related complications
            • Portal vein thrombosis (PVT): Acute or chronic, complete or partial occlusion of portal vein ± extension into mesenteric or splenic veins
              • Local causes
                • Cirrhosis (28%)
                • Abdominal malignancy (27-44%)
                • Inflammatory conditions (10%) (e.g., diverticulitis, pancreatitis, appendicitis, inflammatory bowel disease)
                • Intraabdominal infection
                • Portal vein injury
              • Systemic causes
                • Inherited thrombophilia (20%) (e.g., factor V Leiden, prothrombin mutation)
                • Acquired thrombophilia
                  • Myelodysplastic syndrome (11%)
                  • Others: Pregnancy, hormone use (including oral contraceptive pills), antiphospholipid syndrome, etc.
              • Mortality rates without treatment; justify risk of endovascular treatment after failed anticoagulation
                • < 10%: Chronic PVT in patient without cirrhosis or malignancy
                • 26%: Chronic PVT in patient with cirrhosis &/or malignancy
                • 20-50%: Acute PVT with evidence of bowel ischemia/infarct
            • Clinical presentation
              • Acute thrombosis: Spectrum depends on severity
                • Right upper quadrant tenderness ± hepatomegaly
                • Jaundice, ascites
                • Findings of bowel ischemia/infarction with PVT
                  • Abdominal pain
                  • Nausea, vomiting, diarrhea
                  • Fever, sepsis
              • Chronic thrombosis: Symptoms related to portal HTN
                • Ascites
                • Development of varices ± bleeding
                • Splenomegaly

          PREPROCEDURE

          • Indications

            • Contraindications

              • Preprocedure Imaging

                • Getting Started

                  PROCEDURE

                  • Patient Position/Location

                    • Procedure Steps

                      • Alternative Procedures/Therapies

                        OUTCOMES

                        • Complications

                          • Expected Outcomes

                            Selected References

                            1. Sharma AM et al: Portal vein thrombosis: when to treat and how? Vasc Med. 21(1):61-9, 2016
                            2. Abdel-Aal AK et al: Endovascular treatment of acute portal vein thrombosis using ultrasound-accelerated catheter-directed thrombolysis. Vasc Endovascular Surg. 48(7-8):460-5, 2014
                            3. Wang MQ et al: Acute symptomatic mesenteric venous thrombosis: treatment by catheter-directed thrombolysis with transjugular intrahepatic route. Abdom Imaging. 36(4):390-8, 2011
                            4. Ponziani FR et al: Portal vein thrombosis: insight into physiopathology, diagnosis, and treatment. World J Gastroenterol. 16(2):143-55, 2010
                            5. Liu FY et al: Interventional treatment for symptomatic acute-subacute portal and superior mesenteric vein thrombosis. World J Gastroenterol. 15(40):5028-34, 2009
                            6. Semiz-Oysu A et al: Interventional radiological management of prehepatic obstruction of [corrected] the splanchnic venous system. Cardiovasc Intervent Radiol. 2007 Jul-Aug;30(4):688-95. Erratum in: Cardiovasc Intervent Radiol. 31(1):232, 2008
                            7. Darcy M: Single-session treatment of portal vein thrombosis using combined pharmacomechanical thrombolysis. Semin Intervent Radiol. 24(3):327-32, 2007
                            8. Ferro C et al: Transjugular intrahepatic portosystemic shunt, mechanical aspiration thrombectomy, and direct thrombolysis in the treatment of acute portal and superior mesenteric vein thrombosis. Cardiovasc Intervent Radiol. 30(5):1070-4, 2007
                            9. Kim HS et al: Transhepatic catheter-directed thrombectomy and thrombolysis of acute superior mesenteric venous thrombosis. J Vasc Interv Radiol. 16(12):1685-91, 2005
                            10. Bilbao JI et al: Transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of venous symptomatic chronic portal thrombosis in non-cirrhotic patients. Cardiovasc Intervent Radiol. 27(5):474-80, 2004
                            11. Kumar S et al: Mesenteric venous thrombosis. N Engl J Med. 345(23):1683-8, 2001
                            12. Miller VE et al: Pulsed Doppler duplex sonography and CT of portal vein thrombosis. AJR Am J Roentgenol. 145(1):73-6, 1985
                            Related Anatomy
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                            Related Differential Diagnoses
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                            References
                            Tables

                            Tables

                            KEY FACTS

                            • Terminology

                              • Preprocedure

                                • Procedure

                                  • Post Procedure

                                    TERMINOLOGY

                                    • Definitions

                                      • Hepatic venous outflow obstruction
                                        • 3 categories based on level of obstruction
                                          • Venoocclusive disease: Sinusoids and terminal venules
                                            • Toxin-induced nonthrombotic obstruction; often occurs after stem cell transplant, chemotherapy, or radiotherapy
                                          • Budd-Chiari syndrome (BCS)
                                            • Pain, ascites, liver enlargement caused by hepatic vein occlusion
                                          • Congestive hepatopathy
                                            • Passive hepatic congestion, usually related to right heart failure (e.g., tricuspid regurgitation, cor pulmonale)
                                        • Associated problems of hepatic venous outflow obstruction
                                          • Cirrhosis, portal hypertension (HTN), hepatic/portal vein thrombosis
                                      • Hepatic vein thrombosis (HVT): Acute or chronic, complete or partial occlusion of hepatic veins ± extension into inferior vena cava (IVC)
                                        • Usually due to BCS
                                          • Primary
                                            • Intraluminal thrombosis or webs
                                            • Atypical myeloproliferative disease present in 50%
                                            • Etiologies also include pregnancy, polycythemia vera, Behçet disease, paroxysmal nocturnal hemoglobinuria
                                          • Secondary
                                            • Nonthrombotic; intraluminal invasion (e.g., tumor) or extraluminal compression
                                        • Can lead to portal HTN and related complications
                                      • Portal vein thrombosis (PVT): Acute or chronic, complete or partial occlusion of portal vein ± extension into mesenteric or splenic veins
                                        • Local causes
                                          • Cirrhosis (28%)
                                          • Abdominal malignancy (27-44%)
                                          • Inflammatory conditions (10%) (e.g., diverticulitis, pancreatitis, appendicitis, inflammatory bowel disease)
                                          • Intraabdominal infection
                                          • Portal vein injury
                                        • Systemic causes
                                          • Inherited thrombophilia (20%) (e.g., factor V Leiden, prothrombin mutation)
                                          • Acquired thrombophilia
                                            • Myelodysplastic syndrome (11%)
                                            • Others: Pregnancy, hormone use (including oral contraceptive pills), antiphospholipid syndrome, etc.
                                        • Mortality rates without treatment; justify risk of endovascular treatment after failed anticoagulation
                                          • < 10%: Chronic PVT in patient without cirrhosis or malignancy
                                          • 26%: Chronic PVT in patient with cirrhosis &/or malignancy
                                          • 20-50%: Acute PVT with evidence of bowel ischemia/infarct
                                      • Clinical presentation
                                        • Acute thrombosis: Spectrum depends on severity
                                          • Right upper quadrant tenderness ± hepatomegaly
                                          • Jaundice, ascites
                                          • Findings of bowel ischemia/infarction with PVT
                                            • Abdominal pain
                                            • Nausea, vomiting, diarrhea
                                            • Fever, sepsis
                                        • Chronic thrombosis: Symptoms related to portal HTN
                                          • Ascites
                                          • Development of varices ± bleeding
                                          • Splenomegaly

                                    PREPROCEDURE

                                    • Indications

                                      • Contraindications

                                        • Preprocedure Imaging

                                          • Getting Started

                                            PROCEDURE

                                            • Patient Position/Location

                                              • Procedure Steps

                                                • Alternative Procedures/Therapies

                                                  OUTCOMES

                                                  • Complications

                                                    • Expected Outcomes

                                                      Selected References

                                                      1. Sharma AM et al: Portal vein thrombosis: when to treat and how? Vasc Med. 21(1):61-9, 2016
                                                      2. Abdel-Aal AK et al: Endovascular treatment of acute portal vein thrombosis using ultrasound-accelerated catheter-directed thrombolysis. Vasc Endovascular Surg. 48(7-8):460-5, 2014
                                                      3. Wang MQ et al: Acute symptomatic mesenteric venous thrombosis: treatment by catheter-directed thrombolysis with transjugular intrahepatic route. Abdom Imaging. 36(4):390-8, 2011
                                                      4. Ponziani FR et al: Portal vein thrombosis: insight into physiopathology, diagnosis, and treatment. World J Gastroenterol. 16(2):143-55, 2010
                                                      5. Liu FY et al: Interventional treatment for symptomatic acute-subacute portal and superior mesenteric vein thrombosis. World J Gastroenterol. 15(40):5028-34, 2009
                                                      6. Semiz-Oysu A et al: Interventional radiological management of prehepatic obstruction of [corrected] the splanchnic venous system. Cardiovasc Intervent Radiol. 2007 Jul-Aug;30(4):688-95. Erratum in: Cardiovasc Intervent Radiol. 31(1):232, 2008
                                                      7. Darcy M: Single-session treatment of portal vein thrombosis using combined pharmacomechanical thrombolysis. Semin Intervent Radiol. 24(3):327-32, 2007
                                                      8. Ferro C et al: Transjugular intrahepatic portosystemic shunt, mechanical aspiration thrombectomy, and direct thrombolysis in the treatment of acute portal and superior mesenteric vein thrombosis. Cardiovasc Intervent Radiol. 30(5):1070-4, 2007
                                                      9. Kim HS et al: Transhepatic catheter-directed thrombectomy and thrombolysis of acute superior mesenteric venous thrombosis. J Vasc Interv Radiol. 16(12):1685-91, 2005
                                                      10. Bilbao JI et al: Transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of venous symptomatic chronic portal thrombosis in non-cirrhotic patients. Cardiovasc Intervent Radiol. 27(5):474-80, 2004
                                                      11. Kumar S et al: Mesenteric venous thrombosis. N Engl J Med. 345(23):1683-8, 2001
                                                      12. Miller VE et al: Pulsed Doppler duplex sonography and CT of portal vein thrombosis. AJR Am J Roentgenol. 145(1):73-6, 1985