Portal hypertension

Last updated: November 27, 2023

Summarytoggle arrow icon

Portal hypertension is the pathological elevation of portal venous pressure resulting from obstructions in portal blood flow, which can be prehepatic (e.g., portal vein thrombosis), hepatic (e.g., liver cirrhosis), or posthepatic (e.g., right-sided heart failure). The subsequent backflow of blood may lead to portosystemic anastomoses, splenomegaly, and/or ascites. A diagnosis of portal hypertension can be made based on clinical signs and knowledge of an underlying cause. In suspected cases, medical imaging and laboratory tests are used to support the diagnosis. Management involves treating the underlying condition and reducing portal pressure with nonselective beta blockers (NSBBs) and portosystemic shunts. Acute hemorrhage of esophageal varices is a potentially life-threatening complication of portal hypertension and is caused by increased blood flow via portosystemic anastomoses.

Etiologytoggle arrow icon

Causes of portal hypertension are described based on the anatomical location of the lesion.

Prehepatic [1]

Hepatic [1][4]

Hepatic etiologies of portal hypertension are described anatomically in relation to the hepatic sinusoids.

Posthepatic [1][3]

Clinical featurestoggle arrow icon

The etiology determines whether portal hypertension manifests as acute or chronic.

Patients usually present with signs of their underlying disease and/or symptoms related to the complications of portal hypertension.

Diagnosticstoggle arrow icon

General principles [9][10]

The combination of elevated liver stiffness and thrombocytopenia provides the most reliable estimation of CSPH using noninvasive tests. [9]

Laboratory studies [6][9]

Findings are nonspecific but may further support the diagnosis and help to identify the underlying cause.

Imaging studies [9][11]

Transient elastography [9]

  • Indications: Obtain for all patients with nonconfirmed disease.
  • Findings: Correlate with PLT levels to increase diagnostic yield.
    • CSPH is confirmed with the following findings : [9]
      • Liver stiffness > 25 kPa (regardless of PLT level)
      • Liver stiffness 20–25 kPa (if PLT < 150,000/mm3)
      • Liver stiffness 15–20 kPa (if PLT < 110,000/mm3)
    • CSPH is ruled out if liver stiffness < 15 kPa and platelets ≥ 150,000/mm3. [9]

Transient elastography is the recommended noninvasive test for diagnostic confirmation in patients with suspected portal hypertension. [9]

Abdominal imaging [9]

Findings may help establish the diagnosis and identify the underlying cause.

Hepatic venous pressure gradient (HVPG) measurement [9][10][13]

  • Indication: reserved for diagnostic confirmation in cases of uncertainty (gold standard test) [9]
  • Method: Free and wedge occlusion pressures of the hepatic vein are measured via catheterization, using ultrasound or fluoroscopy. [10]
  • Interpretation of HVPG levels [9]
    • > 5 mm Hg: mild portal hypertension
    • ≥ 10 mm Hg: clinically significant portal hypertension (CSPH) [6]
    • > 12 mm Hg: associated with complications (e.g., variceal bleeding) [6]

Esophagogastroduodenoscopy (EGD) [9][10]

The presence of esophageal varices confirms the diagnosis of CSPH. [9]

Treatmenttoggle arrow icon

General principles [9][10][11]

Consult hepatology for specialist-guided management that includes:

Pharmacotherapy (off-label) [9][10]

Transjugular intrahepatic portosystemic shunt (TIPS or TIPSS) [14][15]

Shunt implementation results in reduced hepatic elimination of ammonia and may lead to worsening of encephalopathy.

Surgical portosystemic shunts [18][19]

Complicationstoggle arrow icon

We list the most important complications. The selection is not exhaustive.

Referencestoggle arrow icon

  1. Nakhleh RE. The pathological differential diagnosis of portal hypertension. Clin Liver Dis (Hoboken). 2017; 10 (3): p.57-62.doi: 10.1002/cld.655 . | Open in Read by QxMD
  2. Singal AK, Kamath PS, Tefferi A. Mesenteric Venous Thrombosis. Mayo Clin Proc. 2013; 88 (3): p.285-294.doi: 10.1016/j.mayocp.2013.01.012 . | Open in Read by QxMD
  3. Garcia-Tsao G. Idiopathic Noncirrhotic Portal Hypertension: What Is It?. Clin Liver Dis (Hoboken). 2015; 5 (5): p.120-122.doi: 10.1002/cld.472 . | Open in Read by QxMD
  4. Schouten JNL, Garcia-Pagan JC, Valla DC, Janssen HLA. Idiopathic noncirrhotic portal hypertension. Hepatology. 2011; 54 (3): p.1071-1081.doi: 10.1002/hep.24422 . | Open in Read by QxMD
  5. Guimarães Cavalcanti M, Marcello de Araujo-Neto J, Mauro Peralta J. Schistosomiasis: Clinical management of liver disease. Clin Liver Dis (Hoboken). 2015; 6 (3): p.59-62.doi: 10.1002/cld.495 . | Open in Read by QxMD
  6. Koh C, Heller T. Approach to the diagnosis of portal hypertension. Clin Liver Dis (Hoboken). 2012; 1 (5): p.133-135.doi: 10.1002/cld.78 . | Open in Read by QxMD
  7. Al-Busafi SA, McNabb-Baltar J, Farag A, Hilzenrat N. Clinical Manifestations of Portal Hypertension. Int J Hepatol. 2012; 2012: p.1-10.doi: 10.1155/2012/203794 . | Open in Read by QxMD
  8. Biecker E. Portal hypertension and gastrointestinal bleeding: Diagnosis, prevention and management. World J Gastroenterol. 2013; 19 (31): p.5035.doi: 10.3748/wjg.v19.i31.5035 . | Open in Read by QxMD
  9. Kaplan DE, Bosch J, Ripoll C, et al. AASLD practice guidance on risk stratification and management of portal hypertension and varices in cirrhosis. Hepatology. 2023.doi: 10.1097/hep.0000000000000647 . | Open in Read by QxMD
  10. de Franchis R, Bosch J, Garcia-Tsao G, et al. Baveno VII – Renewing consensus in portal hypertension. J Hepatol. 2022; 76 (4): p.959-974.doi: 10.1016/j.jhep.2021.12.022 . | Open in Read by QxMD
  11. Simonetto DA, Liu M, Kamath PS. Portal Hypertension and Related Complications: Diagnosis and Management. Mayo Clin Proc. 2019; 94 (4): p.714-726.doi: 10.1016/j.mayocp.2018.12.020 . | Open in Read by QxMD
  12. Garcia‐Tsao G, Abraldes JG, Berzigotti A, et al.. Portal hypertensive bleeding in cirrhosis: Risk stratification, diagnosis, and management: 2016 practice guidance by the American Association for the study of liver diseases. Hepatology. 2016; 65 (1): p.310-335.doi: 10.1002/hep.28906 . | Open in Read by QxMD
  13. Bochnakova T. Hepatic Venous Pressure Gradient. Clin Liver Dis (Hoboken). 2021; 17 (3): p.144-148.doi: 10.1002/cld.1031 . | Open in Read by QxMD
  14. Boike JR, Thornburg BG, Asrani SK, et al. North American Practice-Based Recommendations for Transjugular Intrahepatic Portosystemic Shunts in Portal Hypertension. Clin Gastroenterol Hepatol. 2022; 20 (8): p.1636-1662.e36.doi: 10.1016/j.cgh.2021.07.018 . | Open in Read by QxMD
  15. Lee EW, Eghtesad B, Garcia-Tsao G, et al. AASLD Practice Guidance on the use of TIPS, variceal embolization, and retrograde transvenous obliteration in the management of variceal hemorrhage. Hepatology. 2023.doi: 10.1097/hep.0000000000000530 . | Open in Read by QxMD
  16. Patidar KR, Sydnor M, Sanyal AJ. Transjugular Intrahepatic Portosystemic Shunt. Clin Liver Dis. 2014; 18 (4): p.853-876.doi: 10.1016/j.cld.2014.07.006 . | Open in Read by QxMD
  17. Bhogal HK, Sanyal AJ. Transjugular intrahepatic portosystemic shunt: An overview. Clin Liver Dis (Hoboken). 2012; 1 (5): p.173-176.doi: 10.1002/cld.96 . | Open in Read by QxMD
  18. Taslakian B, Faraj W, Khalife M, et al. Assessment of surgical portosystemic shunts and associated complications: The diagnostic and therapeutic role of radiologists. Eur J Radiol. 2015; 84 (8): p.1525-1539.doi: 10.1016/j.ejrad.2015.04.021 . | Open in Read by QxMD
  19. Harmantas A. Selective vs total portosystemic shunts in the treatment of variceal hemorrhage in cirrhotic patients: is there any advantage?. Hepatology Res. 1999; 14 (2): p.144-153.doi: 10.1016/s1386-6346(98)00121-1 . | Open in Read by QxMD
  20. Boyer TD, Haskal ZJ. The role of transjugular intrahepatic portosystemic shunt in the management of portal hypertension. Hepatology. 2005; 41 (2): p.386-400.doi: 10.1002/hep.20559 . | Open in Read by QxMD

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