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Portopulmonary Hypertension: Symptoms and Causes

Posted on November 09, 2022
Medically reviewed by
Steven C. Pugliese, M.D.
Article written by
Maureen McNulty

Portopulmonary hypertension (POPH) is a condition that leads to high blood pressure in the blood vessels of the liver and the lungs. POPH is serious, but getting quick treatment can help you live longer and improve your quality of life. Read on to learn more about POPH and its symptoms, what causes it, and how it’s treated.

What Is Portopulmonary Hypertension?

Portopulmonary hypertension is related to other conditions with similar-sounding names, including:

  • Pulmonary hypertension (PH) — High blood pressure in the blood vessels in the lungs
  • Pulmonary arterial hypertension (PAH) — A type of PH that develops when the lungs’ blood vessels thicken and narrow (called group 1 PH)
  • Portal hypertension — High blood pressure in the blood vessels of the liver

With POPH, PAH and portal hypertension occur at the same time, affecting both the lungs and the liver.

As many as 15 percent of people with PAH may have POPH. Additionally, POPH may occur in up to 10 percent of people who have portal hypertension.

People with POPH are at risk of developing further liver problems. High pressure in the liver’s blood vessels can cause blood vessels to break open, leading to gastrointestinal bleeding. POPH sometimes occurs with underlying liver disease.

POPH also leads to problems with your lungs and heart. Blood has a more difficult time flowing through the narrowed blood vessels in your lungs, which means the right side of your heart works harder than it should to pump blood. This extra effort damages and weakens your heart. Eventually, POPH may lead to heart failure.

Causes of Portopulmonary Hypertension

Most people who develop POPH have cirrhosis of the liver. This condition occurs when your liver becomes damaged and scarred and stops working properly. Cirrhosis can be caused by drinking a lot of alcohol, as well as by hepatitis infections, diabetes, or drug misuse.

In some cases, POPH is caused by other factors that lead to liver abnormalities, such as:

  • Portal vein thrombosis — A blood clot in the liver’s main vein
  • Autoimmune diseases — Conditions in which the immune system attacks healthy tissues
  • Infections
  • Reactions to certain drugs
  • Birth defects that affect the liver

It’s not entirely clear how POPH develops, but researchers are continuing to learn more.

Normally, blood from your digestive organs — such as your stomach and intestines — travels through your liver on its way back to your heart, which then pumps the blood to your lungs. Your liver helps digest some of the food, nutrients, and medications that you’ve consumed and helps get rid of toxic molecules.

When your liver is damaged due to cirrhosis or other factors, it may develop scarring and stop working properly. This can make it more difficult for blood to flow through your liver. As a result, some blood bypasses your liver and travels from your digestive system directly back to your heart and lungs through new or existing blood vessels.

This could be a problem for a few reasons. For example, when blood doesn’t pass through your liver, bacteria and toxins from the intestines aren’t removed. Instead, they may travel to your lungs and damage the blood vessels there or trigger immune system reactions that cause your vessels to narrow.

Additionally, your liver controls molecules that widen or narrow your blood vessels. When blood doesn’t go through your liver, levels of these molecules become abnormal, which may cause your lungs’ blood vessels to narrow.

Risk Factors for Portopulmonary Hypertension

If you have high blood pressure in your liver, you are more likely to develop POPH if you are female or have a condition called autoimmune hepatitis, according to a study published in the journal Hepatology. Autoimmune hepatitis occurs when your immune system mistakenly attacks your liver. However, if you have portal hypertension due to a hepatitis C infection, you are less likely to have POPH.

Symptoms of Portopulmonary Hypertension

In earlier stages of this medical condition, you may experience:

  • Shortness of breath during physical activity
  • Breathing difficulty when lying down that gets better when you sit or stand
  • Tiredness or weakness
  • Lightheadedness or dizziness
  • Ascites (fluid buildup in the abdomen)
  • Swelling, especially in the feet, ankles, legs, or hands

Many of these symptoms, including shortness of breath, dizziness, and swelling, are also symptoms of pulmonary hypertension. It’s important to have regular health care visits and tell your doctor about any new or changing symptoms so that they can determine if you are developing another condition like POPH.

If POPH becomes more severe, you may start to become short of breath even when you’re resting. You may also faint, cough up blood, or notice chest pain or discomfort.

Diagnosis of Portopulmonary Hypertension

A basic physical exam and blood tests can help your doctor find any potential signs of liver or lung disease.

Your doctor may look for heart problems with tests like an electrocardiogram (a test that measures the heart’s electrical signals), a chest X-ray (an imaging test that helps your doctor see the organs and blood vessels in your chest), or an echocardiogram (an ultrasound of the heart that can estimate pulmonary artery pressures and check for reduced heart function).

A test called right heart catheterization, which involves passing a thin tube through the right part of the heart, can help measure blood pressure in your lungs and allow your doctor to determine how well your heart is pumping.

Pulmonary function tests can help your doctor see how well your lungs work. These tests may involve measuring your breathing capacity or different gases within your lungs.

How Is Portopulmonary Hypertension Treated?

POPH treatment typically aims to treat your symptoms, boost your overall well-being, allow you to become more active, and help you get healthy enough to have a liver transplant. It’s important to get treatment for POPH. You have a better chance of a good outcome and living longer if you use recommended POPH therapies.

Liver transplantation is a common treatment option for portal hypertension or severe liver disease. During this procedure, your liver is surgically removed and replaced with a new liver from a donor. However, if the blood pressure in your lungs is too high, you may not be able to undergo this procedure because it can cause death in more than one-third of people with severe PAH, according to a study in Annals of Thoracic Medicine. If you have POPH, you may need other treatments to reduce blood pressure in your lungs before you have a liver transplant.

Many of the possible options to treat POPH are also common treatments for PAH:

  • Endothelin receptor antagonists like bosentan (Tracleer), ambrisentan (Letairis), and macitentan (Opsumit) could improve high blood pressure in both the lungs and liver and help people with POPH live longer.
  • Prostanoids such as epoprostenol (Flolan) can widen blood vessels.
  • Sildenafil (Revatio), a type of medication known as a phosphodiesterase inhibitor, may allow blood to move through the lungs’ blood vessels more easily.
  • Soluble guanylate cyclase stimulators like riociguat (Adempas) relax and widen the blood vessels.
  • Digoxin (Lanoxin) is a medication that could improve how well your heart works.
  • Oxygen therapy or supplemental oxygen, which involves getting extra oxygen from a tube or face mask, increases low oxygen levels in the body.
  • Diuretics (water pills) can treat liver disease, but they may put extra strain on your heart.

Some treatments that may help people with PAH or PH may not be a good option for those with POPH. For example, anticoagulants (medications that prevent blood clots) and calcium channel blockers (drugs that relax blood vessels and reduce blood pressure) aren’t recommended for people with POPH.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 48,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Has your doctor told you that you have portopulmonary hypertension or other liver problems? If so, how do you manage your POPH and PH? Share your experience in the comments below, or start a conversation by posting on your Activities page.

All updates must be accompanied by text or a picture.
Steven C. Pugliese, M.D. is affiliated with the Hospital of the University of Pennsylvania in Philadelphia, serving as the director of the pulmonary embolism response team, co-director of the comprehensive pulmonary embolism program, and an assistant professor of clinical medicine. Review provided by VeriMed Healthcare Network. Learn more about him here.
Maureen McNulty studied molecular genetics and English at Ohio State University. Learn more about her here.

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