Clinically significant portal hypertension is defined as a portal vein pressure >10 mm Hg, most commonly attributable to liver disease.

• Incidence of portal hypertension is not known.
• Cirrhosis is the most common cause of portal hypertension in the U.S.
• Portal hypertension is developed by >90% of patients with cirrhosis.
• Alcoholic and viral liver diseases are the most common causes of cirrhosis and portal hypertension in the U.S.
• Schistosomiasis is the main cause of portal hypertension outside the U.S.
• Esophageal varices may appear when portal vein pressure rises to >10 mm Hg.
• Variceal hemorrhage is the most serious complication of portal hypertension and may occur when portal pressures rise >12 mm Hg.

• Jaundice
• Ascites (Fig. 1)
• Spider angiomata
• Testicular atrophy
• Gynecomastia
• Palmar erythema
• Dupuytren contracture
• Asterixis (with advanced liver failure)
• Irritability, encephalopathy
• Splenomegaly
• Dilated veins in the anterior abdominal wall
• Venous pattern on the flanks
• Caput medusae (tortuous collateral veins around the umbilicus)
• Hemorrhoids
• Hematemesis
• Melena
• Pruritus

Pathophysiologically caused by:

• Conditions resulting in an increased resistance to flow:
  1. Prehepatic (e.g., portal vein thrombosis, splenic vein thrombosis, congenital stenosis)
  2. Hepatic (e.g., cirrhosis, alcoholic liver disease, primary biliary cirrhosis, schistosomiasis)
  3. Posthepatic (e.g., Budd-Chiari syndrome, constrictive pericarditis, inferior vena cava obstruction, cor pulmonale, tricuspid regurgitation)
• Conditions leading to increase in portal blood flow:
  1. Splanchnic arterial vasodilation accompanying portal hypertension, mediated by local release of nitric oxide
  2. Arterial-portal venous fistulae

Table 1 describes the pathophysiologic changes in portal hypertension, and Table 2 summarizes the etiologies of portal hypertension.

• Ascites from infection, neoplasm, or other inflammatory processes
• Obesity
• Abdominal organomegaly

The workup of portal hypertension includes blood tests and noninvasive imaging studies to determine if the cause of portal hypertension is prehepatic, hepatic, or posthepatic. Ascitic fluid analysis is a key part of the diagnosis.

• Complete blood count with platelets
• Liver function tests with serum albumin
• Prothrombin and partial thromboplastin times
• Hepatitis B surface antigen and antibody
• Hepatitis C antibody
• In selected cases: Iron, total iron-binding capacity, and ferritin; antinuclear antibody, anti-smooth muscle antibodies, antimitochondrial antibody, ceruloplasmin, alpha-1 antitrypsin
• Ascitic fluid analysis: A serum-ascites albumin gradient ≥1.1 mg/dl suggests portal hypertension. Polymorphonuclear cells ≥250 cells/ml or positive Gram stain or culture suggest complicating spontaneous bacterial peritonitis (SBP)

• Duplex-Doppler ultrasound is effective in screening for portal hypertension.
• Less commonly, CT/MRI/MRA scanning (Figs. E2 and E3) or liver-spleen nuclear medicine scanning can be used if the results from ultrasound are equivocal.
• Upper endoscopy is the most reliable test documenting the presence of esophageal varices.

Dietary sodium restriction to generally 2000 mg/day forms the basis of therapy to limit fluid overload.

• For tense ascites, serial large-volume paracentesis (LVP) is generally recommended. The use of albumin infusion (8 to 10 g/L of ascites fluid removed) during LVP >5 L has been shown to reduce the incidence of postparacentesis circulatory dysfunction, although its use remains somewhat controversial.
• IV diuretics, typically furosemide and spironolactone, are used to achieve natriuresis and net negative salt and water balance. Renal function and serum electrolytes are monitored frequently, with transition to an oral regimen for long-term therapy.
• SBP is treated with IV antibiotics directed against enteric bacteria.
• Acute variceal hemorrhage is treated with crystalloid and blood product resuscitation, IV octreotide, terlipressin/vasopressin or somatostatin, and urgent upper endoscopy, often with sclerotherapy or band ligation. Patients with acute variceal hemorrhage should receive empiric antibiotic therapy for SBP.
• Traditionally, a transjugular intrahepatic portosystemic shunt (TIPS) or surgical shunt placement may be considered in patients not responding to above measures. However, recent data show early TIPS placement improved outcomes in acute variceal hemorrhage. Table 3 summarizes indications, contraindications, and complications of the TIPS procedure.
• Table 4 compares treatment modalities for portal hypertension.

• Dietary sodium restriction in combination with diuretics: The typical ratio of furosemide 40 mg to spironolactone 100 mg retains normal serum potassium levels in most patients.
• Nonselective beta-blockers (propranolol and nadolol) in dosages sufficient to reduce the resting heart rate by 25% have been shown to be effective in primary prophylaxis for first-time variceal bleeding and for preventing recurrent variceal bleeding. Dosages are usually given bid and decreased if heart rate falls to <55 beats/min or systolic blood pressure drops to <90 mm Hg. The addition of a long-acting nitrate (e.g., isosorbide-5-mononitrate) has been shown to improve portal hemodynamics. Findings of a prospective trial of beta-blockers to prevent the formation of varices were negative. The combination of beta-blockade plus endoscopic esophageal variceal banding is superior to either intervention alone.
• Intermittent LVP may be needed in “diuretic-resistant” patients.
• Patients with prior SBP merit lifelong antibiotics for secondary prevention.
• Abstinence from alcohol or treatment for hepatitis B or hepatitis C. Vaccination for hepatitis A and B as appropriate.
• Hepatic transplantation is an option in selected patients.

• The most common complication associated with portal hypertension is variceal bleeding. The risk of bleeding from varices is approximately 15% at 1 yr.
• Development of the hepatorenal syndrome (HRS) is associated with high near-term mortality. In particular, HRS may complicate SBP, which emphasizes the importance of making the diagnosis of SBP and instituting appropriate prophylaxis.

Consultation with a gastroenterologist is recommended in all patients with portal hypertension to screen for esophageal varices.

Portal hypertension and its complications carry significant morbidity and mortality rates. Emphasize ethanol abstinence, provide vaccinations and prophylactic therapy where indicated, and consider early referral to a specialist for assistance with management and consideration for hepatic transplantation.