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 generally progressive, with risk of complications including acute variceal bleeding, ascites, hepatic encephalopathy, and hepatorenal syndrome.

Epidemiology

Incidence
The exact incidence of portal hypertension is unclear.

Prevalence

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

Etiology and Pathophysiology

• Causes generally classified as follows:
  • Prehepatic (portal vein thrombosis or obstruction)
  • Intrahepatic (most commonly cirrhosis)
  • 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 cirrhosis (PBC)
  • Sarcoidosis
• Increased HVPG results in venous collateral formation in the distal esophagus, proximal stomach, rectum, and umbilicus.
• Gastroesophageal variceal formation is found in 40% of patients with portal hypertension.
• Progression of portal hypertension results in splanchnic vasodilation and angiogenesis.

Genetics
No known genetic patterns except those associated with specific hepatic diseases that cause portal hypertension

Risk Factors

See “Etiology and Pathophysiology.”

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

General Prevention

Treatment of the underlying liver disease may reduce portal hypertension and prevent complications in patients with cirrhosis.

Commonly Associated Conditions

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