Pulmonary Arterial Hypertension Overview

Pulmonary arterial hypertension (PAH) is continuous high blood pressure in the pulmonary artery. The pulmonary artery carries blood from the right side of the heart to small arteries in the lungs. In PAH, the arterial walls tighten, thicken and scar. This leads to narrowing of the artery. In addition, tiny blood clots may cause blockage of blood flow. All of these changes cause the right side of the heart to work harder to pump blood to the lungs. Over time, the heart weakens and cannot pump blood adequately. This may lead to right heart failure.

PAH may be primary or secondary. Primary PAH, a rare condition, is inherited or the cause is unknown. Secondary PAH can be linked to another condition, such as blood clots in the lung or a lung disease.

Some patients have no symptoms until the condition is serious. The most common symptoms include difficulty breathing, dizziness or fainting spells. Other symptoms include cyanosis (bluish color of the skin), fatigue (feeling tired), and swelling of the ankles. Symptoms worsen with high altitudes, air travel and pregnancy.

Although there is no cure for PAH, many treatment options are available. Treatment is based on the specific problem causing your condition. Medications that relax and open up the arteries to allow better circulation include bosentan, epoprostenol, treprostinil, and sildenafil. Additional treatments may include anticoagulants (blood thinners), calcium channel blockers and digitalis. Diuretics (fluid pills) and oxygen are often used to relieve symptoms. Surgery is indicated only when required for removal of blood clots or, in severe cases, for lung or heart-lung transplant. 

Living with Pulmonary Arterial Hypertension

Healthy Pointers

To learn more about pulmonary arterial hypertension, please visit http://www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_all.html

 

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