Portal Hypertension



  • Increased portal venous pressure >5 mm Hg that occurs in association with splanchnic vasodilatation, portosystemic collateral formation, and hyperdynamic circulation
  • Most commonly secondary to elevated hepatic venous pressure gradient (HVPG; the gradient between portal and central venous pressures)
  • Course is progressive, with risk of acute variceal bleeding, ascites, hepatic encephalopathy, portopulmonary syndrome, and hepatorenal syndrome.



  • Prevalence: <200,000 persons in the United States
  • Predominant sex: male > female adults

Etiology and Pathophysiology

  • Develops as a consequence of resistance to blood flow in the portal venous system
  • Causes generally classified as follows:
    • Prehepatic (portal vein thrombosis or obstruction)
    • Intrahepatic (most commonly cirrhosis or schistosomiasis)
    • Posthepatic (hepatic vein thrombosis, Budd-Chiari syndrome, right-sided heart failure)
  • 90% of intrahepatic cases are due to cirrhosis secondary to the following:
    • Virus (hepatitis B, hepatitis C, hepatitis D)
    • Alcoholism
    • Nonalcoholic fatty liver disease
    • Schistosomiasis
    • Wilson disease
    • Hemochromatosis
    • Primary biliary cholangitis (PBC)
    • Sarcoidosis
  • Increased HVPG results in venous collateral formation in the distal esophagus, proximal stomach, rectum, and umbilicus.
  • Progression of portal hypertension results in splanchnic vasodilation and angiogenesis.
  • Gastroesophageal variceal formation is found in 40% of patients with portal hypertension.

Risk Factors

Pediatric Considerations
Portal vein thrombosis is the most common extrahepatic cause; intrahepatic causes are more likely to be biliary atresia, viral hepatitis, and metabolic liver disease.

Commonly Associated Conditions

  • Alcoholism
  • Cirrhosis
  • Nonalcoholic fatty liver disease
  • Schistosomiasis
  • Extrahepatic portal vein thrombosis

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