What Is Pulmonary Arterial Hypertension?

Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a serious, life-threatening condition. The condition gradually gets worse over time and can lead to heart failure. Treating the condition can help to decrease symptoms to improve the life of those who have the condition.

What Is Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension (PAH) is a rare type of pulmonary hypertension. It is not the same as high blood pressure, and it is sometimes referred to as type 1 pulmonary hypertension. When a person has PAH, the small blood vessels (pulmonary arteries) that are responsible for transporting blood through the lungs begin to narrow. This narrowing restricts the blood flow and causes blood pressure levels to rise. The heart works harder to pump the blood into these arteries. This can eventually weaken the heart and cause heart failure.

There are several reasons that PAH may develop. These reasons include:

  • Blood clots in lungs
  • Congestive heart failure
  • Congenital heart defects
  • Eisenmenger syndrome
  • HIV
  • Chronic disease of the liver or cirrhosis
  • Illegal drug use, such as methamphetamines or cocaine
  • Autoimmune diseases, such as lupus or rheumatoid arthritis
  • Glycogen storage disorders
  • Sleep apnea

Sometimes there is no reason for PAH to develop. When this occurs, the condition is known as idiopathic PAH.

Idiopathic Pulmonary Arterial Hypertension Symptoms

Idiopathic pulmonary arterial hypertension symptoms gradually worsen over time. When the condition occurs, the symptoms may be so slight that they aren’t noticed and will become increasingly problematic as the disease progresses. These symptoms include:

  • Shortness of breath
  • Dizziness
  • Swelling in the legs and ankles
  • Weakness
  • Chest pains
  • Passing out or fainting
Diagnosing Pulmonary Arterial Hypertension

When a patient presents with the symptoms of PAH, the doctor will order tests to confirm the diagnosis. These tests may include:

  • Ultrasounds of the heart
  • CT scans
  • Ventilation-perfusion scan
  • Electrocardiogram
  • Exercise testing
  • Chest x-ray
Pulmonary Arterial Hypertension Medications

With new scientific advances, there are more pulmonary arterial hypertension medications and treatment options than ever before.

  • Blood thinners help to decrease the risk of blood clots by preventing blood cells from sticking to each other.
  • Calcium channel blockers decrease high blood pressure both inside and outside of the lungs. They work by preventing calcium from entering the muscle cells in the heart and the arteries. This decreases the workload of the heart and decreases blood pressure levels.
  • Diuretics can be used if swelling occurs. Diuretics may be prescribed to remove the excess fluid from the legs and ankles.
  • Prostacyclin-based therapies are a type of PAH medicine that mimics prostacyclin, a substance the body naturally produces. Prostacyclin widens the blood vessels to improve the flow of blood and increase oxygen supply.
  • Phosphodiesterase-5 inhibitors are another type of medication that is only for PAH. This PAH medicine works by blocking an enzyme known as phosphodiesterase-5. Blocking this enzyme results in the widening of the pulmonary arteries.
  • Endothelin is a substance that can cause the blood vessels to narrow. Endothelin receptor antagonists block endothelin’s action, which makes the blood vessels relax and widen.

Though there is currently no cure for PAH, beginning treatment as soon as the condition is diagnosed can help lengthen the life and improve the life quality for those with the disease. Talk with a doctor about PAH medicines that may be right for you.


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