Portal Hypertension

Background

• Portal hypertension is a syndrome of increased pressure (> 5 mm Hg) in the portal venous system due to increased vascular resistance plus increased blood flow.
• It is most commonly a complication of cirrhosis and should be suspected in all patients with cirrhosis, but there are many other possible causes.
• The condition may be asymptomatic, but also can present with:
  • variceal hemorrhage (must be urgently excluded in any patient with suspected liver disease presenting with significant gastrointestinal hemorrhage)
  • splenomegaly
  • ascites
  • abdominal pain (may be present in extrahepatic portal vein obstruction).

Evaluation

• Suspect portal hypertension in all patients with cirrhosis. Tests that support the diagnosis include:
  • imaging studies, such as upper gastrointestinal endoscopy and Doppler ultrasound
  • transient elastography to measure liver stiffness
  • serum-ascites albumin gradient (SAAG) which is calculated as the serum albumin concentration minus the ascitic fluid albumin concentration (suspect portal hypertension if ≥ 1.1 g/dL)
• Definitive diagnosis requires direct measurement of portal venous pressure, but this is invasive and requires significant procedural experience (balloon catheterization into hepatic vein via femoral or jugular route) (Strong recommendation).
• Portal hypertension is diagnosed if hepatic venous pressure gradient (HVPG) is > 5 mm Hg; clinically significant portal hypertension is HVPG ≥ 10 mm Hg (Strong recommendation).

Management

• Management of portal hypertension differs by the etiology of underlying disease, stage of disease if cirrhosis is involved, and degree of portal hypertension which may be clinically reflected by the presence of complications. The primary goal of treatment is to prevent and treat complications, especially variceal hemorrhage, clinically apparent ascites, and extrahepatic portal vein obstruction.
• Perform esophagogastroduodenoscopy (EGD) to diagnose esophageal and gastric varices at the time of cirrhosis diagnosis (Strong recommendation) and every 3 years in patients with compensated cirrhosis (Strong recommendation) or annually if hepatic decompensation occurs (Strong recommendation).
• Screening for varices can be deferred in patients with liver stiffness measurement < 20 kPa from transient elastography and platelet count > 150,000 per mm³ as they have a very low probability (< 5%) of having high-risk varices.
• For patients with varices that have not bled (primary prophylaxis of variceal hemorrhage):
  • Use nonselective beta blockers for primary prophylaxis of variceal hemorrhage if medium or large varices (> 5 mm) are present (Strong recommendation) and if small varices are present and the patient is at increased risk of hemorrhage (Strong recommendation).
  • Use at starting dose 6.25 mg twice daily or lower starting dose if hypotensive. Less preferred alternatives include traditional beta blockers Propranolol at starting dose 20 mg twice daily or at 40 mg once daily.
  • EGD follow-up is not necessary in patients taking beta blockers (Strong recommendation).
  • Endoscopic variceal ligation (variceal band ligation) is recommended as an option for prevention of the first variceal hemorrhage in patients with medium or large varices (> 5 mm) that have not bled (Strong recommendation) or in decompensated patients who have not tolerated beta blockers.
  • Repeat ligation every 2-8 weeks until obliteration (Strong recommendation).
• For preventing rebleeding after variceal hemorrhage episode (secondary prophylaxis):
  • Use a combination of nonselective beta blockers plus endoscopic variceal ligation for secondary prophylaxis of variceal hemorrhage (Strong recommendation).
  • Start secondary prophylaxis as soon as possible following index variceal bleeding episode when hemodynamically able to tolerate (Strong recommendation).
  • Repeat ligation every 1-4 weeks until obliteration (Strong recommendation).
  • Use transjugular intrahepatic portosystemic shunt (TIPS) for recurrent variceal bleeding despite the combination of pharmacologic and endoscopic therapy (Strong recommendation).
  • Perform pre-emptive TIPS within 72 hours in patients with bleeding varices who are Child-Pugh class C < 14 points or Child-Pugh class B > 7 with active bleeding at initial endoscopy (Strong recommendation).