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 either inherited or occurs for no known reason. Secondary PAH can be linked to another condition, such as blood clots in the lung or a lung disease.
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What are the symptoms?
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.
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What treatments are available?
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 Tracleer® (bosentan), Flolan® (epoprostenol), Remodulin® (treprostinil), and Revatio® (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.
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How can I maximize my therapy?
- Always take medications as directed. Do not skip doses and never change your dose unless instructed by your doctor.
- Learn about side effects of your medication, and when it is important to inform your doctor of adverse effects.
- Use oxygen only as directed.
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Healthy Pointers
- Eat a healthy diet, avoid smoking, and practice relaxation techniques.
- Discuss exercise and activity recommendations with your doctor.
- Women of childbearing age should discuss birth control with their doctor.
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Where can I find more information?
To learn more about PAH, please visit: www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_all.html
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