Treating and Managing Pulmonary Arterial Hypertension

How Pulmonary Arterial Hypertension Is Treated

Although there is no cure for PAH, there are medications and procedures that can slow the progression of the disease and improve your quality of life. Studies indicate that early diagnosis is associated with improved survival rates and will help with getting started on the right treatment path. Treatment options vary from person to person, so you will want to work closely with your healthcare provider to determine the best plan for you. 

Newly diagnosed patients with PAH should be referred to an accredited pulmonary hypertension care center for thorough evaluation and right heart catheterization. Because PAH is such a rare disease, it is extremely valuable to see a specialist at an accredited center to ensure you are getting the most up-to-date treatment options. 

PAH-specific medications come in multiple forms: oral, inhaled and intravenous (IV)/subcutaneous.

Intravenous treatments open up the blood vessels to help ease chest pain and shortness of breath. Subcutaneous treatments use a portable infusion pump to open up the blood vessels and ease PH symptoms. Inhaled treatments, such as prostacyclin, are primarily used to relieve shortness of breath.

There are a wide range of oral treatment options. Endothelin Receptor Antagonists (ERAs) prevent blood vessels from narrowing and are commonly used to help patients participate in physical activities. Phosphodiesterase Inhibitors (PDE 5 Inhibitors) allow the lungs to relax and the blood vessels to dilate. Prostacyclin Analogue, Selective IP Receptor Agonist, and Soluble Guanylate Cyclase (sGC) Stimulators allow the blood vessels in the lungs to relax.

A number of supportive therapies are recommended in conjunction with medication. Diuretics can help treat fluid retention and swelling. Anticoagulants may be prescribed to prevent blood clots from forming. Some patients may require a portable oxygen tank. In the most severe cases, a heart-lung, single lung or double lung transplant may be recommended. Pulmonary rehabilitation as well as daily exercise are also highly recommended to help patients improve breathing and quality of life.

Treatment aims to restore balance among one or more of three substances that are produced by your lungs: nitric oxide, endothelin, and prostacyclin. Although a test does not currently exist to determine which of these substances is not balanced, PAH medications act on these three pathways to help slow how quickly your disease worsens.

Managing Pulmonary Arterial Hypertension

Being diagnosed with a chronic illness like PAH is life-changing. You will need to have a close relationship with a PAH expert and would benefit from a strong support system of family and friends.

Many pulmonary hypertension care centers require visits every few months and regular testing such as echocardiograms and 6-minute walk testing. Some centers will do right heart catheterizations every year to see how well treatment is working on managing pulmonary pressures and heart function. Additionally, it is essential to take your medications exactly as directed, being careful not to run out or change your schedule unless directed by your healthcare provider. It is also important to stay up-to-date on all recommended vaccinations.

Patients with PAH should adopt or continue these lifestyle changes to maintain their health.

  • Quitting smoking and avoiding secondhand smoke is one of the most important decisions you can make. The American Lung Association offers great resources to help you quit. 
  • Though PAH may make you feel fatigued, it is important to stay active and exercise as much as possible. You may need to avoid activities such as weightlifting that can put too much strain on your lungs and heart. Ask your healthcare provider for help creating a safe exercise plan.
  • Avoid sitting in a hot tub or sauna, as well as traveling to high altitudes, as that can also put strain on your lungs and heart.
  • Eating healthy will be an important part of managing your disease. Many healthcare providers suggest avoiding saturated fat, trans fat and cholesterol and limiting your salt intake.
  • Pregnancy can put strain on your body and for a patient with PAH be possibly life-threatening, so this is a subject you should discuss with your healthcare provider prior to becoming pregnant.    
  • With all this change, patients with PAH can easily feel overwhelmed. Many individuals may experience anxiety and/or depression after diagnosis. There are a number of support groups and other mental healthcare options available. If you are having problems coping with your situation, talk to your healthcare provider about getting some help.

To compensate for the blocked blood flow through your lungs, the right side of the heart works harder to pump blood through the narrowed arteries.1

Right heart failure can occur due to increased stress on your heart2

The most common cause of death in PAH is right heart ventricular failure1

The outcome for untreated PAH is poor.1

The mean survival of untreated IPAH is 2 to 3 years from the diagnosis.1

References:

  1. Pahal P, Sharma S. Idiopathic Pulmonary Artery Hypertension. [Updated 2022 Apr 14]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482251
  2. Montani, D., Günther, S., Dorfmüller, P. et al. Pulmonary arterial hypertension. Orphanet J Rare Dis 8, 97 (2013). https://doi.org/10.1186/1750-1172-8-97

Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel.

Page last updated: October 26, 2023

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