Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension (PAH) is a rare and serious condition that affects the lungs and heart, it occurs when the blood vessels in the lungs become narrowed and blocked or destroyed, making it difficult for the heart to pump blood through them.

PAH is a rare disease, it can be life-threatening if left untreated, so in this article, we will delve into the causes, symptoms, diagnosis, and treatment options for PAH, as well as lifestyle changes and coping strategies for those living with this condition.

Whether you are someone living with PAH, or simply interested in learning more about this condition, this article will provide a comprehensive guide to help you better understand PAH.


What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary Arterial Hypertension (PAH) is a rare and serious condition that affects the blood vessels in the lungs and heart, it occurs when the small arteries in the lungs become narrowed and blocked or destroyed, making it difficult for the heart to pump blood through them.

PAH leads to increased pressure in the pulmonary artery, which is the blood vessel that carries blood from the heart to the lungs to pick up oxygen.

As the pressure in the pulmonary artery increases, the heart has to work harder to pump blood through the lungs, which can eventually lead to damage to the heart and other organs.

PAH can also cause blood clots to form in the lungs, which can further worsen the condition.

It is a chronic and progressive disease that can be life-threatening if left untreated, and it is a rare disease, with an estimated prevalence of around 15-50 cases per million adults worldwide.

The condition is more common in women than men, and it typically affects people between the ages of 20 and 60.

There are several forms of PAH, including idiopathic (no known cause), hereditary, and associated with other medical conditions such as connective tissue diseases, HIV infection, and liver disease.


Causes of PAH

The cause of pulmonary hypertension is not always clear. Certain medical conditions can damage, change, or block the blood vessels of the pulmonary arteries, which can lead to pulmonary hypertension.

Some examples of medical conditions include:

Left heart diseases, such as left heart failure, which may be caused by high blood pressure throughout your body or coronary heart disease.

Other heart and blood vessel diseases such as congenital (inherited) heart defects.

Lung diseases such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, emphysema, or sleep apnea.

Other medical conditions such as liver disease, sickle cell disease, blood clots in the lungs, or connective tissue disorders like scleroderma.




Several factors can increase your risk of developing pulmonary hypertension.

Here are some of the known causes and risk factors of PAH:



  • Age: Pulmonary hypertension can occur at any age, but your risk increases as you get The condition is usually diagnosed between ages 30 and 60.
  • Environment: You may be at an increased risk of pulmonary hypertension if you have or are exposed to Asbestos or certain infections caused by
  • Family history and genetics: Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can increase your risk of pulmonary A family history of blood clots also increases your risk.
  • Lifestyle habits: Unhealthy lifestyle habits such as smoking and illegal drug use can raise your risk of developing pulmonary
  • Medicine: Some prescribed medicines used to treat cancer and depression may increase your risk of pulmonary


  • Sex: Pulmonary hypertension is more common in women than in Pulmonary hypertension with certain types of heart failure is also more common in women.


note that: having one or more of these risk factors does not necessarily mean that a person will develop PAH.

However, if you have any of these risk factors and are experiencing symptoms such as shortness of breath, chest pain, or fatigue, it’s important to speak with your healthcare provider.


Pulmonary Arterial Hypertension Symptoms

PAH can cause a range of symptoms that can vary depending on the severity of the condition, The symptoms of PAH can be nonspecific and may be mistaken for other conditions, which can make diagnosis difficult. Here are some of the most common signs and symptoms of PAH: Shortness of breath: This is one of the most common symptoms of PAH and may occur during physical activity or even at rest.

Fatigue: People with PAH may experience fatigue or weakness due to the heart having to work harder to pump blood through the lungs.

Chest pain: This is another common symptom of PAH and may be experienced as a sharp or dull pain in the chest.

Rapid heartbeat: People with PAH may experience a rapid or irregular heartbeat, also known as palpitations.

Dizziness or fainting: PAH can cause a decrease in blood flow to the brain, which may result in dizziness or fainting.

Swelling in the legs or ankles: PAH can cause fluid to build up in the legs and ankles, resulting in swelling.

Bluish lips or skin: This is a sign of low oxygen levels in the blood, which can occur in advanced cases of PAH.

Wheezing: which is a whistling sound when you breathe out.

Coughing that is dry or produces blood.



It’s important to note that: these symptoms can also be caused by other conditions, and not everyone with PAH will experience all of these symptoms.

If you are experiencing any of these symptoms, it’s important to speak with your healthcare provider, early diagnosis and treatment can help to manage the symptoms of PAH and improve your quality of life.


What are the treatment options for PAH?


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 doctor  to determine the best plan for you.


There are several treatment options available for Pulmonary Arterial Hypertension (PAH), although there is currently no cure for the disease. The goal of treatment is to manage the symptoms of PAH, improve quality of life, and slow the progression of the disease.


Here are some of the most common treatment options for PAH: Medications: There are several medications available that can help to reduce the pressure in the pulmonary artery and improve blood flow.

These medications include vasodilators, calcium channel blockers, and endothelin receptor antagonists, some of these medications may be taken orally, while others may be delivered through an injection or infusion.

Oxygen therapy: People with PAH may also benefit from oxygen therapy, which can help to improve oxygen levels in the blood and reduce the workload on the heart.

Pulmonary rehabilitation: Pulmonary rehabilitation is a program of exercise and education designed to improve lung function and overall physical fitness.

It may be recommended for people with PAH to help them manage their symptoms and improve their quality of life.

Surgery: In some cases, surgery may be recommended to improve blood flow through the lungs, this may include procedures such as balloon pulmonary angioplasty, pulmonary thromboendarterectomy, or lung transplant.

Lifestyle changes: Making certain lifestyle changes can also help to manage the symptoms of PAH, these may include quitting smoking, maintaining a healthy weight, and avoiding high altitudes, which can exacerbate symptoms.

note that: treatment for PAH may need to be tailored to each individual, depending on the severity of the condition and other factors such as age and overall health.

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Fawaz Almutairi

Saudi Arabia

Fawaz Portrait GIS president


President, Gulf Intervention Society

Interventional Cardiologists

National Guard Hospital

Riyadh, Saudi Arabia

Abdullah Shehab

United Arab Emirates

shehab portrait vice president GIS


Vice President, Gulf Intervention Society

Professor of Cardiovascular Medicine

Chairman of Education, EMA 

Editor Chief New Emirates Medical Journal 

Alain, Abu Dhabi, UAE

Khalid Bin Thani



Treasurer, Gulf Intervention Society

Head of Scientific Committee, GIS Conference

Consultant Interventional Cardiologist

Bahrain Specialist Hospital Manama, Bahrain
Khalid Bin Thani GIS

Mousa Akbar



General Secretary, Gulf Intervention Society

Head of Cardiology Unit, Al Sabah Hospital

Kuwait City, Kuwait

mousa Akbar GIS