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Portal hypertension

Last updated: April 26, 2021

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Portal hypertension refers to a pathological elevation of portal venous pressure resulting from obstructions in portal blood flow, which may be either prehepatic (e.g., portal vein thrombosis), hepatic (e.g., liver cirrhosis), or posthepatic (e.g., right-sided heart failure). The subsequent backflow of blood may lead to portosystemic anastomoses, splenomegaly, and/or ascites. While portal hypertension may be diagnosed purely based on the presence of clinical signs and potential risk factors, medical imaging and laboratory tests are used to support the diagnosis in suspected cases. Management requires treating the underlying condition and reducing portal pressure with nonselective beta-blockers and portosystemic shunts.

A potentially life-threatening complication is acute hemorrhage of the esophageal varices caused by increased blood flow via portosystemic anastomoses. Patients present with sudden hematemesis and melena, as well as hypovolemic shock in some cases. In addition to stabilizing the patient, acute management of variceal bleeding includes reducing splanchnic blood flow with octreotide and endoscopic variceal band ligation. Prevention of (recurring) bleeding involves nonselective beta-blockers, endoscopic variceal ligation, or placement of transjugular intrahepatic portosystemic shunts (TIPS).

  • Portal hypertension is defined as a hepatic venous pressure gradient (HVPG) of ≥ 6 mm Hg.
  • HVPG > 10 mm Hg is clinically significant and > 12 mm Hg is associated with complications.

References:[1]

Depending on the cause, portal hypertension may be either acute or chronic. Acute portal hypertension arises from acute portal vein thrombosis, while chronic portal hypertension may be due to chronic thrombosis, cirrhosis, or schistosomiasis.

References:[2]

Clinical manifestations of portal hypertension (e.g., ascites) in a patient with a known risk factor (e.g., cirrhosis) may already suffice for diagnosis. In addition to investigating underlying conditions, diagnostic steps may include:

References:[3]

Medical therapy

Beta-blocker treatment in patients suffering from advanced liver cirrhosis (Child-Pugh class C) may lead to circulatory dysregulation. If negative effects outweigh the benefits, beta-blocker treatment should be reconsidered!

Portosystemic shunts

References:[4][5]

Esophageal variceal hemorrhage

Definition

Esophageal variceal hemorrhage; refers to the bleeding of dilated sub-mucosal veins (varices) of the distal esophagus; and is a dangerous consequence of portal hypertension. It is the most common form of upper gastrointestinal (GI) bleeding in patients presenting with cirrhosis.

Clinical features

Clinical diagnosis

  • Sudden onset of severe upper GI bleeding in a patient with signs of portal hypertension, typically in combination with liver failure
  • If bleeding occurs following retching or vomiting, consider a Mallory-Weiss tear as a differential diagnosis.

Treatment

Acute management of variceal hemorrhage

  • Resuscitation and stabilization
    • Bring patient into shock position
    • Place (at least) two peripheral venous catheters
    • Substitute crystalloids to maintain plasma volume
    • Intubate patient (with decreased consciousness) to prevent the possibility of aspiration or airway obstruction
    • Place a stomach tube → helps to remove the blood
    • Intensive care monitoring
    • Transfuse blood or blood products to stabilize coagulation, if indicated
  • Medical therapy
  • Endoscopic management
    • Erythromycin (a strong prokinetic agent) may be administered before gastroscopy.
    • Procedures
      • Endoscopic band ligation (procedure of choice)
        • Hemostasis
        • Used for primary prophylaxis and prevention of recurring hemorrhage
      • Alternative: injection sclerotherapy, absolute alcohol, and fibrin glue, as well as cyanoacrylate, to stop acute variceal bleeding
      • Balloon tamponade using a Sengstaken-Blakemore tube or Minnesota tube
        • Indication: alternative treatment in case of extreme hemorrhage, unsuccessful endoscopic treatment, or ineffective hemostatic medication; consider in hemodynamically unstable patients until they can be stabilized
        • Complication: risk of decubitus gangrene → prevention: deflation of balloon every 5 hours for 5 minutes
  • Interventional radiologic treatment: See “TIPS” above.

Bleeding prevention

Other complications

References:[6][7][8]

We list the most important complications. The selection is not exhaustive.

  1. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  2. Le T, Bhushan V. First Aid for the USMLE Step 1 2015. McGraw-Hill Education ; 2014
  3. Carale J. Portal Hypertension. Portal Hypertension. New York, NY: WebMD. http://emedicine.medscape.com/article/182098. Updated: November 20, 2016. Accessed: March 29, 2017.
  4. Sanyal AJ, Bajaj JS. Transjugular intrahepatic portosystemic shunts: Indications and contraindications. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/transjugular-intrahepatic-portosystemic-shunts-indications-and-contraindications?source=search_result&search=TIPS&selectedTitle=1~150.Last updated: November 7, 2016. Accessed: February 12, 2017.
  5. Sanyal AJ. Primary and Pre-primary Prophylaxis Against Variceal Hemorrhage in Patients with Cirrhosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/primary-and-pre-primary-prophylaxis-against-variceal-hemorrhage-in-patients-with-cirrhosis.Last updated: November 2, 2015. Accessed: January 8, 2018.
  6. Bajaj JS, Sanyal AJ. Methods to achieve hemostasis in patients with acute variceal hemorrhage. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://stage0www.uptodate.com/contents/methods-to-achieve-hemostasis-in-patients-with-acute-variceal-hemorrhage?source=search_result&search=octreotid&selectedTitle=11~150.Last updated: November 12, 2015. Accessed: February 12, 2017.
  7. Reynaert H, Geerts A. Pharmacological rationale for the use of somatostatin and analogues in portal hypertension. Aliment Pharmacol Ther. 2003; 18 (4): p.375-386.
  8. Sanyal AJ. General principles of the management of variceal hemorrhage. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/general-principles-of-the-management-of-variceal-hemorrhage#H10.Last updated: December 22, 2016. Accessed: February 12, 2017.