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Portal hypertension is an increase in the pressure within the portal vein, which carries blood from the digestive organs (stomach, intestine, spleen, & pancreas) to the liver. The increase in pressure is caused by a blockage in the blood flow through the liver.

Increased pressure in the portal vein causes large veins (varices) to develop across the esophagus and stomach to get around the blockage. The varices become fragile and can bleed easily.

Many times, people suffering from portal hypertension do not realize that there is a problem until they experience an episode of pain on one side of their body. This could be in the form of a dull ache or throbbing pain that spreads across the lower portion of the arm, or perhaps it happens when you flex your wrist.

Causes

The most common cause is cirrhosis (scarring) of the liver, but thrombosis (clotting) might also be the cause.

  • Cirrhosis is scarring which accompanies the healing of liver injury caused by hepatitis, alcohol, or other less common causes of liver damage. In cirrhosis, the scar tissue blocks the flow of blood through the liver.
  • Other causes include blood clots in the portal vein, blockages of the veins that carry the blood from the liver to the heart, a parasitic infection called schistosomiasis, and focal nodular hyperplasia, a disease seen in people infected with HIV, the virus that may lead to AIDS. Sometimes the cause is unknown.

Symptoms

  • Gastrointestinal bleeding: You may notice blood in the stools, or you may vomit blood if any large vessels around your stomach that developed due to portal hypertension rupture.
  • Ascites: When fluid accumulates in your abdomen, causing swelling
  • Increased spleen size (splenomegaly)
  • Encephalopathy, or confusion and fogginess in thinking
  • Jaundice, the yellowing of the skin and the whites of the eyes
  • Edema, (swelling) of the legs
  • Caput medusa, a visible network of dilated veins surrounding your navel
  • Swollen veins on the anterior abdominal wall (sometimes referred to as caput medusae)

Diagnosis

  • Imaging studies-duplex Doppler ultrasound (uses sound waves to see how the blood flows through the portal vein)
  • Blood tests
  • Pressure measurement studies
  • Endoscopic diagnosis
    • During a gastrointestinal endoscopy, the doctor can see the mucous lining of the upper gastrointestinal tract, including the esophagus, stomach and duodenum (first part of the small intestine).

Complications

  • 'Ascites'- excess fluid in your abdominal cavity
    • Early feeling of fullness
    • Increase in size of abdomen
    • Feeling out of breath (if the fluid begins pushing on your lungs)
  • Varices - varicose veins associated with portal hypertension
    • Varices most often occur in the esophagus or stomach as a result of portal hypertension. This is often because the liver tissue is scarred and blood cannot flow through normally. As the portal blood is rerouted due to the increased resistance, varices develop.
  • Encephalopathy - impairment in neuropsychiatric function associated with portal hypertension
    • Symptoms are usually mild, with subtle changes in behavior, changes in sleep pattern, mild confusion & forgetfulness or slurred speech. However, it can progress to more serious symptoms, including severe lethargy and coma.
  • Reduced levels of platelets, blood cells that help form blood clots, or white blood cells, the cells that fight infection.

Treatment

  • Unfortunately, most causes of portal hypertension cannot be treated. Instead, treatment focuses on preventing or managing the complications, especially the bleeding from the varices. Diet, medications, endoscopic therapy, surgery, and radiology procedures all have a role in treating or preventing the complications. Other treatment depends on the severity of the symptoms and on how well your liver is functioning.

Prognosis / Mortality & Morbidity

  • Without treatment, portal hypertension can lead to severe complications, such as chronic bleeding, abdominal swelling, and liver failure. Doctors typically treat portal hypertension with a combination of blood pressure-lowering medication, lifestyle changes, and surgery.

From: https://www.medscape.com/answers/182425-95873/what-are-the-mortalit... Medscape - What are the mortality and morbidity of portal vein obstruction? By: Adnan Said, MD, MSPH; Chief Editor: BS Anand, MD, Jul 09, 2020]

  • In the absence of cirrhosis, the 2-year bleeding risk from esophageal varices is reported to be 0.25% and of those that bleed the mortality rate is approximately 5%. Those with cirrhosis and varices have a 20-30% 2 year bleeding risk with a mortality rate of 30-70%. This difference is primarily a consequence of the normal hepatic function in the noncirrhotic patient. The variceal size is the major predictive factor for bleeding.
  • In adults with portal vein thrombosis, the 10-year survival rate has been reported to be 38-60%, with most of the deaths occurring secondary to the underlying disease (eg, cirrhosis, malignancy).

From: NIH – Natural history of portal hypertension in patients with cirrhosis. By R de Franchis & M Primignaani

  • All patients with cirrhosis will eventually develop portal hypertension and esophagogastric varices. Bleeding from ruptured esophagogastric varices is the most severe complication of cirrhosis and is the cause of death in about one third of patients.
    • The rate of development and growth of esophageal varices is poorly defined but in general seem to be related to the degree of liver dysfunction.
    • Once varices have formed, they tend to increase in size and eventually to bleed. In unselected patients, the incidence of variceal bleeding is about 20% to 30% at 2 years.
  • Variceal size is the single most important predictor of a first variceal bleeding episode.
  • Portal hypertensive gastropathy can progress from mild to severe and vice-versa or even disappear completely. Acute bleeding from portal hypertensive gastropathy seems to be relatively uncommon, and less severe than bleeding from varices.

Resources & Additional reading

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