Portopulmonary hypertension is a progressive complication of high blood pressure in the veins that lead to your liver. It eventually causes right sided heart failure.
Portopulmonary hypertension is characterized by high blood pressure in the arteries that carry blood to your lungs.
Its name can be broken into three parts:
- “Porto” refers to the veins that bring blood from many different internal organs to your liver.
- “Pulmonary” refers to arteries that supply your lungs.
- “Hypertension” is the medical term for high blood pressure.
People with portopulmonary hypertension may develop symptoms like shortness of breath or fatigue as the disease progresses. It can eventually lead to right sided heart failure, especially if it’s left untreated.
Read on to learn more about portopulmonary hypertension, including its symptoms, causes, and outlook.
Potential signs and symptoms include:
- shortness of breath, especially with exertion
- fatigue
- ascites, fluid buildup in your abdomen
- bulging jugular vein in your neck
- chest pain
- heart palpitations
- lightheadedness
- dizziness
- low blood oxygen
Portopulmonary hypertension is a subtype of pulmonary arterial hypertension, which is high blood pressure in the arteries that bring blood to your lungs.
By definition, portopulmonary hypertension is a complication of portal hypertension. Portal hypertension is high blood pressure in the veins that supply your liver. The most common cause of portal hypertension is cirrhosis.
The development of portosystemic shunts can put more pressure on the arteries leading to your lungs or expose them to harmful substances normally filtered by your liver.
About
Portopulmonary hypertension is the third most common cause of pulmonary arterial hypertension based on data from France. It’s thought to make up about 5–10% of cases, but some newer reports have increased this estimate to 15%.
Portopulmonary hypertension is thought to occur in about 2–10% of people with portal hypertension.
Potential
- female sex
- autoimmune hepatitis
- elevated estradiol levels
- spleen removal (splenectomy)
- portosystemic shunts
- anemia
Portopulmonary hypertension is a progressive complication of portal hypertension. People with portopulmonary hypertension eventually develop right sided heart failure.
Right sided heart failure means the right side of your heart can no longer efficiently pump blood. Symptoms of right sided lung failure include:
- shortness of breath
- chest pain
- heart palpitations
- fluid retention or swelling in your lower body and abdomen
Doctors start the diagnosis of portopulmonary hypertension by:
- considering your symptoms
- reviewing your personal and family medical history
- performing a physical exam
If a doctor suspects a problem with your heart or lungs, they may order other tests, such as:
- hepatic venous pressure gradient, which involves inserting a long tube called a catheter into a blood vessel
- right heart catheterization
- liver elastography
- transthoracic echocardiography
- blood tests
- tests to measure your breathing such as spirometry
Treatment for portopulmonary hypertension is broken into
- general measures to avoid complications such as:
- avoiding pregnancy
- yearly immunization against influenza and pneumococcal disease
- supportive treatments such as:
- specific medications to improve the outlook of people with the disease, such as:
- prostacyclin agonists
- endothelin receptor antagonists
- phosphodiesterase inhibitors
- guanylate cyclase stimulants
- prostacyclin agonists
Liver transplant is a potential option for people who respond to treatment to lower blood pressure in their pulmonary arteries.
The French Pulmonary Hypertension Registry reported the following survival rates from people with portopulmonary hypertension from the years 2007–2017:
| Years after diagnosis | Survival rate |
|---|---|
| 1 year | 84% |
| 3 years | 69% |
| 5 years | 51% |
The outlook for people with portopulmonary hypertension is very poor if left untreated. Only about 14% of people survive at least 5 years without treatment.
The severity of cirrhosis seems to be a predictor of the chances of survival.
Portopulmonary hypertension isn’t always preventable because some causes are out of your control. Some ways you can lower your likelihood of developing portal hypertension include:
- avoiding exposure to
asbestos - avoiding smoking
- avoiding using illegal drugs
- managing high blood pressure and coronary heart disease
- receiving treatment for chronic liver disease
- minimizing alcohol intake
It’s important to get medical attention if you have potential symptoms of pulmonary hypertension, such as trouble breathing or unexplained fatigue. It’s also critical to go to all your follow-up appointments with a doctor if you’ve previously received a diagnosis of portal or pulmonary hypertension.
Here are some frequently asked questions people have about portopulmonary hypertension.
What is the pulmonary pressure for portopulmonary hypertension?
Having an average pulmonary arterial pressure greater than 20 millimeters of mercury (mm Hg) is one of the diagnostic criteria for portopulmonary hypertension.
What is the difference between portopulmonary hypertension and hepatopulmonary syndrome?
Portopulmonary hypertension is pulmonary hypertension associated with high blood pressure in the veins that lead to your liver. Hepatopulmonary syndrome is reduced blood oxygen levels due to the relaxation of the blood vessels in your lung in the presence of advanced liver disease.
Portopulmonary hypertension is a complication of portal hypertension characterized by increased blood pressure in the arteries that supply your lungs with oxygen. It’s a progressive condition that eventually causes right sided heart failure.
Portopulmonary hypertension may not cause symptoms until it causes complications. The outlook for people with portopulmonary hypertension can be improved by taking medications to control the progression of the disease.