• Clinical science

Portal hypertension

Abstract

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).

Etiology

Portal venous pressure of > 10 mm Hg (normal value: 3–6 mm Hg) is considered pathological. Causes of portal hypertension can be classified as follows:

References:[1]

Clinical features

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]

Diagnostics

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]

Treatment

Medical therapy

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

Portosystemic shunts

References:[4][5]

Complications

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.

Paquet grading of esophageal varices

Grading of esophageal varices, modified by Paquet
Grade I Varices surpass level of surrounding tissue, disappear with insufflation
Grade II Enlarged, straight varices (≤ 1/3 of esophageal lumen), which do not disappear with insufflation
Grade III More prominent, coil-shaped varices (≤ 1/2 of esophageal lumen), which partially touch one another
Grade IV Almost complete obstruction of esophageal lumen; varices are located even in the upper third of the esophagus

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 Cyanoacrylate is particularly useful if varices are located at the transition to the gastric fundus.
      • Balloon tamponade using a Sengstaken-Blakemore tube or Minnesota tube
        • Indication: variceal bleeding in the cardia or lower part of the esophagus; 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

  • Primary prophylaxis
    • Medication to lower portal pressure, irrespective of variceal grading: nonselective beta-blockers (e.g., propranolol)
    • Endoscopic esophageal variceal ligation: only indicated for patients with a high risk of bleeding (cirrhosis Child B–C, coagulopathy or varices Paquet grade II)
  • Secondary prophylaxis
    • Combine endoscopic variceal ligation and medication (e.g., propranolol) to reduce portal pressure for residual varices
    • If bleeding occurs despite secondary prophylaxis → placement of TIPS

Other complications

References:[6][7][8]

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