Portopulmonary hypertension is increased arterial pressure in the vessels of the lungs and the vein that delivers blood to the liver.

Portopulmonary hypertension involves high blood pressure in the arteries in the lungs and the portal vein. The portal vein delivers blood from the intestines to the liver.

High blood pressure can develop separately in the lungs and portal vein. Doctors diagnose portopulmonary hypertension when both types of high blood pressure happen together.

Experts do not know exactly how many people live with this type of hypertension. Estimates suggest it accounts for anywhere from 5–15% of cases of high blood pressure in the lungs. It also affects about 5–6% of people with advanced liver disease.

This article covers the symptoms, causes, and treatments of portopulmonary hypertension.

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Before understanding portopulmonary hypertension, it may be helpful to learn a bit about pulmonary hypertension and portal hypertension.

Pulmonary hypertension

Damage, scarring, or narrowing of the vessels in the lungs can cause an increase in blood pressure in the vessels of the lungs.

This type of high blood pressure is uncommon. Only about 500–1,000 people receive a pulmonary hypertension diagnosis in the United States each year.

Common causes of high blood pressure in the lungs include:

Portal hypertension

Portal hypertension involves high blood pressure in the portal venous system. The portal venous system delivers blood to the liver.

The most common cause of this type of high blood pressure is liver cirrhosis, also known as advanced liver disease.

Additional causes of portal hypertension include:

  • a blood clot in the vein
  • sarcoidosis — an autoimmune disease
  • toxicity from medications

Doctors categorize the condition as portopulmonary hypertension when a person has high blood pressure in the portal vein and arteries of the lungs.

Portopulmonary hypertension tends to have a worse outlook and survival rate than pulmonary hypertension.

According to a 2020 review, a poor outlook may be more likely when a person has liver disease and portopulmonary hypertension.

Symptoms of portopulmonary hypertension are similar to the symptoms of pulmonary hypertension.

However, since it often develops due to liver disease, a person may also experience other symptoms, such as:

  • shortness of breath
  • fatigue
  • weakness
  • chest pain
  • fainting
  • heart palpitations
  • coughing
  • dizziness

Portopulmonary hypertension often develops due to cirrhosis.

Over time, structural changes in the liver can cause alterations in the veins that may lead to problems with blood flow. This increases blood pressure in the portal veins, causing portal hypertension.

When portal hypertension develops, it also affects other areas of the body.

For example, the heart has to work harder, which may affect the vessels in the lungs, leading to portopulmonary hypertension.

In addition to advanced liver disease, the condition can also develop due to:

  • a blood clot in the portal vein
  • drug reactions
  • infections
  • congenital abnormalities

Factors that increase the risk of liver disease also increase the chances of developing portopulmonary hypertension. They include:

  • heavy alcohol use
  • methamphetamine use
  • prior history of some types of chemotherapy
  • certain autoimmune diseases

Doctors diagnose portopulmonary hypertension on the basis of a physical exam, symptom review, and diagnostic tests.

During a physical exam, doctors look for signs of leg swelling, heart murmur, and jugular vein distension (bulging of the major veins in the neck).

About 40 in 100 people with portopulmonary hypertension have a tricuspid valve murmur.

The standard diagnostic test to confirm a diagnosis of portopulmonary hypertension is a procedure known as right heart catheterization.

Diagnosis of portal hypertension

To diagnose portal hypertension, doctors assess the clinical features of the condition using abdominal ultrasound and CT scans, including:

  • Ascites: This is when fluid gathers in the abdomen.
  • Varices: This means the veins in the esophagus are too big.
  • Splenomegaly: This term means the spleen is enlarged.
  • Encephalopathy: This refers to any brain condition that can change the structure or function of the brain.

Treatment of portopulmonary hypertension remains similar to treating general pulmonary hypertension. The goal of treatment is to lower blood pressure in the arteries of the lungs.

Usually, treatment involves medications known as vasodilators to widen the blood vessels, which decreases blood pressure in the arteries.

Liver transplantation can help improve the condition. However, having portopulmonary hypertension may exclude a person from getting a liver transplant.

Portopulmonary hypertension is increased blood pressure in the portal veins that deliver blood to the liver and the pulmonary arteries in the lungs.

It typically develops due to severe liver disease.

Symptoms of portopulmonary hypertension include fatigue, chest pain, and shortness of breath, especially with activity.

Treatment involves medication that can help reduce pressure in the blood vessels.