Portal hypertension (portal HTN) is defined as increased pressure in the portal system due to increased resistance of portal blood flow. This most commonly affects adults and children with cirrhosis or advanced fibrosis of the liver. Noncirrhotic portal HTN is a rarer entity caused by a variety of conditions, the most common being hepatic schistosomiasis.

The most common etiologies of cirrhosis / advanced fibrosis are nonalcoholic steatohepatitis, chronic viral hepatitis (mostly B and C), alcoholic hepatitis, and hemochromatosis.

Clinical manifestations of portal HTN include porto-systemic shunting (varices), splenomegaly, and thrombocytopenia. Symptoms of underlying cirrhosis such as jaundice may present first. Symptoms of portal HTN typically do not occur until complications arise. These complications can include ascites, spontaneous bacterial peritonitis (SBP), hepatorenal syndrome (HRS), hepatic hydrothorax, hepatopulmonary syndrome, portal hypertensive gastropathy, gastroesophageal variceal bleed, and hepatic encephalopathy. If the diagnosis is in doubt, the hepatic venous pressure gradient (HVPG) can be measured for confirmation. This represents the gradient between the pressure of the portal vein compared to the inferior vena cava (IVC), which is usually between 1-5 mm Hg.

Early diagnosis of portal HTN is key, as late manifestaptions of disease can be life-threatening and can result in severe decompensation in patients with cirrhosis.

Related topic: Portopulmonary hypertension