Description

• 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.

Epidemiology

Prevalence

• 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