Pulmonary Arterial Hypertension (PAH) – Symptoms, Causes And Treatment


What Is PAH?

One of the most brutal forms of Hypertension or blood pressure is known as Pulmonary Arterial Hypertension (PAH). PAH comes under the category of Pulmonary Hypertension and mainly occurs due to the narrowing and contraction of blood vessels.

The fact that it is rare, speaks about the immense danger it carries along. These pulmonary arteries are responsible for carrying blood to and from the heart and due to the formation of extra tissues in the arteries. The path for blood flow becomes narrow and there is increased blood flow, leading to Hypertension or high blood pressure.

PAH can be labeled as a progressive disorder, which indicates that initially, the patient may experience mild uneasiness and pain. But over time, severe medical treatment would be needed to maintain a livable lifestyle.

PAH causes the heart to work much harder due to clogged arteries towards the lung, the extreme pressure, and force used by the heart. It eventually causes the heart muscles to overexert and become weak, and the patient can succumb to heart failure.

1. Symptoms

  • Acute shortness of breath and fatigue during the most basic of physical activities like bending down, walking down the staircase, or even getting up from the bed.
  • Fainting may occur because the lung’s ability to pump blood crosses its threshold due to the thinning of pulmonary arteries via the formation of extra tissues.
  • Edema, also known as the abnormal swelling in the legs or lungs due to excessive fluids. This occurs because the heart does not allow sufficient pumping of blood and the excess fluids’ land up collecting in one spot which leads to swelling in the hands and feet.
  • Fast heart rate which is not controlled by rest. The right ventricle is responsible for pumping the blood that has already gone through the whole body and now has to go to the lungs. Due to clogged arteries linked to the lungs, the right ventricle must apply immense pressure for blood to reach the lungs. This drastically increases heart attack and can mean you are a patient suffering from Pulmonary Arterial Hypertension (PAH).

2. Causes

The widespread cause of PAH is unknown and doctors say the primary cause can be genetic or environmental factors. Greenhouse gases, carbon monoxide and harmful chemicals being released into the air have led to a bunch of health problems for the majority of the world population.

But with detailed research, experimentation, and real-world data, medical practitioners have found some of the causes that may lead to this devastating condition.


Those who are infected by the HIV virus are highly prone to lung diseases. Harmful bacteria and proteins such as Nef and Tat present in the HIV virus, that infects the pulmonary vascular and lung artery cells.

The body then loses its availability to repair arteries and the damaged blood vessels within, which makes the patient work and experience problems such as dyspnea, chronic cough, and swollen legs.

2.2. Usage Of Illegal Drugs And Substances

MDMA, Methamphetamine and Alkaloidal Cocaine consumption often results in cardiopulmonary symptoms. These drugs are a mixture of various combustion products, hydrochloride acid, lidocaine, mannitol, quinine, aspirin, and corn starch that behaves in a volatile manner when consumed via the nose or via.

2.3. Chronic Bronchitis And Emphysema

Discolored mucus forms during inflammation of the bronchial tube and causes respiratory tract infections which leads to persistent cough and breathing difficulties.

The constant loss of capillaries causes the pulmonary artery pressure to periodically rise and force the heart to perform at an unreasonably high intensity which, over time, leads to strokes and heart failures.

2.4. Liver Cirrhosis

Alcohol addiction or insufficient drinking of water causes the liver cells to be replaced by scarred tissues. Once the liver begins to lose these cells, it fails to eradicate toxins from the bloodstream and produce enzymes.

That helps digest food and store beneficial nutrients.

This means there is a higher undesired intake of toxic alien particles and bacteria that damages the delicate small arteries responsible for the flow of blood to the lungs and from the heart.

Just imagine what would happen to your internal organs if the master organ responsible for maintaining flushing out of the harmful carbohydrates and fats is dysfunctional.

3. Treatment

For this form of Hypertension, natural remedies do not make a difference as there is an overgrowth of tissues amongst arteries.

Most relevant medicines help in dilating or opening up the blood vessels present in the lungs to increase blood flow, and also in allowing the lungs to produce extra vasodilators of its on.

The best medications that one can take for PAH are mentioned below:

Oral Treatment Medicines

Inhaled Treatment Medicines

Intravenous Treatment Medicines






Room Temperature stable Epoprostenol



Intravenous Trepostinil








  • In the case of oral treatments, Endothelin Receptor Antagonists (ERAs) help prevent the blood vessels from narrowing. Phosphodoitsterase Inhibitors (PDE 5 Inhibitors) enables the lungs with functionality to create more vasodilators. Prostacyclin allows the blood vessels to relax, and also Solube Guanylate Cyclase (sGC). Stimulators increase the syncing of sGC with nitric oxide in the blood vessels to help them calm down.
  • In the case of inhaled treatment options, the prostacyclins clear the pipes and permit the relieving of shortness of breath.
  • In the case of intravenous treatment options, the blood vessels open up and ease the symptoms of PAH. Relieving the patient of chest pain in the process.


Earlier, patients suffering from PAH did not have many treatment options, due to which their life expectancy reduced considerably. But now, due to innovative solutions and implementation of advanced studies and medicinal procedures, the life expectancy has drastically improved. One thing is confirmed, that PAH can never be reversed or completely cured.

Prescribed treatment may help prevent permanent damage to the pulmonary arteries, which are responsible for pumping blood to the lungs. In case pulmonary arterial hypertension (PAH) is caused by some other issue, then the medical practitioner will treat the underlying cause to help you get rid of PAH in the process.