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Pulmonary Arterial Hypertension: Everything You Need to Know

Pulmonary arterial hypertension refers to a serious health condition that involves your heart and lungs. It’s characterized by high blood pressure in the arteries that go from your heart to your lungs. Keep reading to understand more about this condition and determine if you need to see your doctor.

Pulmonary arterial hypertension (PAH) is a medical condition that can damage the right side of your heart. It occurs when the blood pressure between your heart and lungs is higher than normal. It can happen on its own or be a consequence of another disease.

Hypertension increases the pressure of the blood vessels in your lungs. This means your heart has to work harder to pump blood into them and becomes stressed. Symptoms of PAH include shortness of breath, lightheadedness, and chest pains. If left untreated, PAH can seriously damage your heart muscle. Further complications include arrhythmia (irregular heart rhythm) and heart failure.

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When you have pulmonary hypertension, the walls of the arteries leading from your heart to your lungs become stiff and thick, which means they can’t expand and contract as easily to allow blood through. The reduced blood flow makes it harder for the right side of the heart to pump blood, and when that side has to work harder with each beat, it gets weaker. 

While PAH can affect anyone of any age, it’s most common in people who already have lung disease or a heart condition.

You may not realize that you have PAH, especially in the early stages. When you start noticing symptoms like the ones below, the disease may be more advanced and potentially life-threatening. Because many symptoms aren’t specific to PAH, you may not realize that you have the disease. In addition, diagnosing this condition may be delayed because the symptoms are similar to the underlying cause of hypertension. For people who are younger than 36 or have an underlying medical condition that causes hypertension, pulmonary arterial hypertension may be harder to spot and diagnose.

Symptoms include:

  • Dizziness and fainting spells
  • Shortness of breath both during exercise and when resting
  • Chest pain and pressure
  • Fatigue and listlessness
  • Swelling in your legs, ankles, and abdomen (edema)
  • Racing pulse and heart palpitations
  • Bluish color in your lips and skin (cyanosis)
  • Weight gain from edema or weight loss due to abdominal swelling that diminishes your appetite
  • Uncommon symptoms of PAH include cough and hoarseness due to the pressure on the nerves leading to the vocal cords.

This condition can occur on its own or develop alongside a preexisting heart or lung condition. Some underlying causes can include certain types of congenital heart disease, coronary artery disease, connective tissue disease, cirrhosis of the liver, high blood pressure, blood clots in the lungs, and chronic breathing disorders like emphysema.

There are five different types of pulmonary hypertension, grouped according to their cause.

  • Group 1 pulmonary arterial hypertension is known as idiopathic pulmonary arterial hypertension. Doctors diagnose this type of PAH when the cause is unknown. There’s also a specific gene mutation that causes the condition to develop in families, which is known as heritable pulmonary arterial hypertension. Certain drugs, like prescription weight-loss drugs and amphetamines, can also cause this condition. PAH from heart abnormalities, like congenital heart disease and other chronic conditions, also falls into this group.
  • Group 2 PAH is caused by left-sided heart disease. This includes left-side valvular heart disease and failure of the lower left heart chamber.
  • Group 3 pulmonary hypertension is caused by lung disease, like pulmonary fibrosis, chronic obstructive pulmonary disease, sleep apnea, or long-term exposure to thin air at high altitudes.
  • Group 4 PAH is caused by chronic blood clots.
  • Group 5 PAH is caused by blood disorders, sarcoidosis (which affects many organs in the body), metabolic disorders like glycogen storage disease, and tumors that cause pressure on the pulmonary arteries.

There is another type of disease that causes pulmonary hypertension, called Eisenmenger syndrome. This is caused by a hole in the heart between the ventricles that causes the blood to circulate abnormally in the heart. The oxygen-rich blood and oxygen-poor blood mix together. Then, the blood returns to your lungs rather than the rest of your body, further increasing the pressure in the pulmonary arteries and thus causing hypertension.

There are a few common risk factors that may cause you to develop PAH. If you’re a younger adult, idiopathic pulmonary arterial hypertension is more common. A family history of the disease also increases your risk, as you can develop a heart or lung condition that leads to pulmonary hypertension.

If you’re overweight or have otherwise poor health habits, your risk of developing PAH is higher. Drugs like speed, cocaine, and appetite suppressants can also lead to the condition, as can smoking. Your environment also contributes to your risk of developing pulmonary hypertension. People who live at high altitudes or in homes with asbestos insulation or silica have a higher risk of developing the condition. If you contract a tapeworm, you can also develop an infection that results in PAH.

A family history of the condition increases your risk. Down syndrome and congenital heart disease increase the risk of pulmonary hypertension. Blood clotting disorders, kidney disease, hepatitis B and C, scoliosis, cirrhosis of the liver, and surgical removal of the spleen are other health conditions that can lead to PAH.

Several medications can cause pulmonary hypertension as well. These include:

  • Selective serotonin reuptake inhibitors, which are prescription drugs used to treat depression and anxiety
  • Certain kinds of chemotherapy medications
  • Weight-loss drugs like phen-fen or other appetite suppressants

Pulmonary hypertension is more common in women than in men, as is PAH with a certain kind of heart failure.

PAH can’t be cured, but with proper medical care, you can manage your condition. This can slow the progression of the disease and may make your symptoms more manageable. Some pulmonary arterial hypertension treatments include treating the underlying cause of hypertension, and it may take some trial and error before you and your doctor find a treatment that works for you. With proper treatment, the life expectancy of people with pulmonary arterial hypertension can be quite long. 

Treatment for PAH may include certain medications, like blood vessel dilators (vasodilators), to open narrowed blood vessels. These are administered either through a nebulizer or through an intravenous catheter. Another type of medication is endothelin receptor antagonists, which reverse your level of endothelin, a peptide in the walls of your blood vessels that causes them to narrow. Certain erectile dysfunction drugs are also used to treat PAH as they have the same effect of opening the blood vessels in the body.

Other types of medication you might take for PAH include:

  • High-dose calcium blockers
  • Anticoagulants to prevent the formation of small blood clots in your pulmonary blood vessels
  • Soluble guanylate cyclase stimulator to relax the pulmonary arteries
  • Digoxin to make the heart pump more strongly and pump more blood
  • Diuretics, or water pills, to eliminate excess fluid from your body
  • Oxygen therapy

If medications don’t work, your doctor may recommend surgery such as open-heart surgery or a lung and/or heart transplant. 

If you experience any of the above-described symptoms, visit your doctor. Also, if you know that you have a medical condition that can lead to PAH, you may wish to have your doctor examine your lungs and arteries.

Pulmonary hypertension affects the arteries in your lungs and the right side of the heart. The condition can get progressively worse and can be life-threatening. Although treatment may help to manage PAH, it’s often better to prevent the condition from developing if possible.

PAH can be avoided by making certain lifestyle changes. Quitting smoking, reducing salt in your diet, and avoiding diet pills and street drugs can all help lower your risk of developing PAH.

https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/diagnosis-treatment/drc-20350702

https://www.nhlbi.nih.gov/health-topics/pulmonary-hypertension

https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

https://www.nhs.uk/conditions/pulmonary-hypertension/causes/

https://www.cdc.gov/heartdisease/pulmonary_hypertension.htm

https://www.uptodate.com/contents/clinical-features-and-diagnosis-of-pulmonary-hypertension-of-unclear-etiology-in-adults?search=Pulmonary%20Arterial%20Hypertension&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2

https://www.nhs.uk/conditions/pulmonary-hypertension/

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