Pulmonary Arterial Hypertension (PAH)

Pulmonary Arterial Hypertension (PAH)

What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary arterial hypertension (PAH) is a life-threatening condition that gets worse over time, but treatment can help improve symptoms and improve living with the disease., many are finding ways to do all the things they love, just like they used to. have been diagnosed.

Pulmonary arterial hypertension (PAH) means high blood pressure in the arteries leading from the heart to the lungs. different from normal hypertension


Doctors may not be able to determine the cause of high blood pressure in the lungs. In this case, the condition is called idiopathic pulmonary arterial hypertension (PAH). Genes may play a role in people get it.

If not, another condition is causing the problem. Each of these diseases can lead to high blood pressure in the lungs.

  • Congestive heart failure
  • Thrombi in the lungs
  • HIV
  • Use of illicit drugs (such as cocaine or methamphetamine)
  • Liver disease (such as cirrhosis)
  • lupus, scleroderma, rheumatoid arthritis and other autoimmune diseases
  • Congenital heart defect
  • Lung diseases such as emphysema, chronic bronchitis and pulmonary fibrosis
  • Sleep Apnea


You may not notice any symptoms for a while. The main one is shortness of breath during activity. It usually starts slowly and gets worse over time. You may find that some of the things you used to do now cause you to be out of breath.

Other symptoms are:

  • Chest pain
  • Faintness
  • Faint
  • Ankle and leg swelling

Get a Diagnosis

If you are short of breath and you see a doctor, they will ask about your medical history. They may also ask you:

  • Do you smoke?
  • Does anyone in your family have heart or lung disease?
  • When did your symptoms start?
  • What causes symptoms to get better or worse?
  • Will my symptoms ever go away?

Your doctor may order tests such as:

Echocardiogram: This ultrasound image of a beating heart can check the blood pressure in the pulmonary arteries.

CT scan: may show enlarged pulmonary arteries. A CT scan can also detect other lung problems that may cause shortness of breath.

Ventilation Perfusion Analysis (V/Q Analysis): This test detects blood clots that cause high blood pressure in the lungs.

Electrocardiogram (EKG or ECG): The ECG tracks heart activity and can show if the right side of the heart is working hard. It is a warning sign of pulmonary arterial hypertension (PAH).

Chest x-ray: An x-ray can show if the arteries or heart are enlarged. A chest X-ray can help detect other lung and heart conditions that may be causing problems.

Stress testing: Your doctor may recommend running on a treadmill or riding a stationary bike while hooked up to a monitor to see changes in things like oxygen levels, heart function, and lung pressure.

Your doctor may also perform blood tests to check for conditions such as HIV, rheumatoid arthritis, and lupus.

If these tests indicate the possibility of pulmonary arterial hypertension (PAH), doctors should perform a right heart catheterization. Here’s what happens during this test:

  • Doctors insert a catheter into a large vein, most often the jugular vein in the neck or the femoral vein in the leg, on the right side of the heart.
  • The monitor records the pressure in the right side of the heart and in the pulmonary arteries.
  • Doctors may also inject medication into the catheter to see if the arteries in the lungs are stiff. This is called a vasoreactivity test.

Treatment of Pulmonary arterial hypertension (PAH)

Because Pulmonary arterial hypertension (PAH) is individual, treatment plans are tailored to the patient’s needs. Talk to your doctor about your options and what to expect.

First, your doctor will treat the cause of your condition. For example, if emphysema is causing problems, it should be treated to improve pulmonary hypertension.

Most people also receive treatment to improve their breathing. This makes everyday activities and jobs easier. Breathing pure oxygen through nose-fitting spikes, oxygen therapy helps with shortness of breath and low blood oxygen levels. Helps you live longer when you have pulmonary arterial hypertension (PAH). Doctors recommend blood thinners if there is a risk of blood clots. Other drugs improve the work of the heart and prevent fluid accumulation.

If you have severe Pulmonary arterial hypertension (PAH), your doctor may prescribe drugs called calcium channel blockers. These drugs lower blood pressure in the lungs and other parts of the body.

If calcium channel blockers aren’t enough, your doctor can refer you to a specialized treatment center. You may need more targeted treatments that can open narrowed blood vessels. These may be tablets, inhaled drugs, or drugs given through an IV. Options are:

Pills: Ambrisentan (Letairis), Bosentan (Tracleer), Macitentan (Opsumit), Riociguat (Adempas), Sildenafil (Cenforce 150 mg), Tadalafil (Vidalista 60 mg), Treprostinil (Orenitram)

Inhalers: iloprost tromethamine (Ventavis), treprostinil (Tyvaso)

IV drugs: epoprostenol sodium (Floralan, Velletri), treprostinil

In more serious cases, or when drugs don’t work, doctors may recommend a procedure called lung transplantation or atrial septostomy. A surgeon creates an opening between the right and left sides of the heart. This surgery can have serious side effects.