What is pulmonary arterial hypertension?

Pulmonary arterial hypertension (PAH) is an uncommon but serious medical condition. In PAH, the arteries that carry blood to your lungs are narrow, making blood flow difficult.

Blood travels from your heart to your lungs through arteries. When the blood reaches your lungs, it picks up oxygen and carries it to the rest of your body. When PAH causes those arteries to narrow, pressure increases greatly to help move the blood through the narrowed arteries. High blood pressure, or hypertension, in the pulmonary arteries causes the right side of your heart to work harder and eventually weaken.

Idiopathic PAH is PAH that has no obvious cause. PAH can also be an inherited disease. If you have a family history of PAH, your odds of developing the condition are higher.

PAH most commonly results from other health problems, such as:

  • heart failure
  • congenital heart defects
  • a blood clot in the lungs
  • chronic obstructive pulmonary disease

The symptoms of PAH usually don’t appear until the disease is advanced. One early symptom is shortness of breath and fatigue after minor physical exertion. You may have moments of dizziness or even fainting spells because PAH can diminish blood flow to your brain.

PAH causes your heart to strain to circulate blood throughout your body properly. This can cause fluid to build up. If you have PAH, you may have ankle swelling.

See your doctor if you notice any of these symptoms.

No cure is available for PAH. The treatment focuses on relieving symptoms and slowing the progression of the disease. Your doctor may prescribe medications that can help relax blood vessels or reduce excess cell growth within your arteries.

Some drugs doctors commonly prescribe to treat PAH include sildenafil, prostanoids, and calcium channel blockers. One drug, digoxin, helps the heart beat more strongly. You may also need to take a diuretic, which helps lower blood pressure by reducing fluid levels in your body. Also, your doctor may prescribe drugs to help treat medical conditions that lead to pulmonary hypertension.

Atrial septostomy is a procedure that your doctor can perform to improve blood flow in your heart. During the procedure, your doctor guides a catheter through an artery to your heart’s upper chambers, called the atria. The catheter enters through the septum, the thin wall that separates the right atrium and left atrium.

Your doctor inflates a tiny balloon at the catheter’s tip after insertion to allow more blood flow between the heart’s two chambers. This eases the growing pressure in the right atrium that PAH causes.

People with the most serious cases of PAH may need a lung transplant. This is mostly an option for people whose PAH is the result of a serious lung disease. Lung transplants are for people who may only have a short time to live and who haven’t found relief with medications or other procedures.

Patients who also have severe heart disease or heart failure due to PAH may be candidates for a heart-lung transplant.

Sometimes, a small blood clot in the lungs dissolves on its own. But, if you have a blood clot in the pulmonary artery, a surgeon may need to remove it. A pulmonary thromboendarterectomy is necessary when a blood clot is stuck to the inside of the pulmonary artery, blocking blood flow.

This procedure may cause blood pressure in the pulmonary artery to return to a healthy range. It may also help undo some of the damage to the right side of the heart.

Researchers continue to find new ways to prevent and treat PAH, but you can do your part to reduce your risk of complications from this serious disease.

Along with standard PAH treatments, these lifestyle changes are also important:

  • Take your medications to control your blood pressure and cholesterol levels.
  • Eat a healthier diet.
  • Exercise regularly.
  • Quit smoking if you smoke
  • Lose weight if you’re overweight or obese.

See your doctor to address changes in your condition.