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What Is Pulmonary Hypertension?

Pulmonary hypertension is high blood pressure in the pulmonary artery. The pulmonary artery is the blood vessel that takes oxygen-poor blood from the right side of the heart into the lungs. Blood in the lungs picks up oxygen and flows back to the left side of the heart and the rest of the body. The average pulmonary artery pressure is about 14 mmHg at rest. Hypertension is pressure higher than 25 mmHg at rest or 30 mmHg during exercise. Pulmonary hypertension is primary or secondary. Primary pulmonary hypertension (PPH) is a very rare and serious condition. More women than men have it, usually by age 30 to 40. Secondary pulmonary hypertension occurs in people with other diseases. It’s more common than PPH.

What Causes Pulmonary Hypertension?

The cause of PPH isn’t known. People with secondary pulmonary hypertension often have severe pulmonary disease, COPD (emphysema or chronic bronchitis), or blood clots (pulmonary emboli) in the lungs.

What Are the Signs and Symptoms of Pulmonary Hypertension?

The first symptom is often tiredness. Breathing problems (dyspnea), dizziness, and fainting are also seen early. Ankle or leg swelling (edema), bluish lips and skin, and chest pain (angina) are other possible manifestations of pulmonary hypertension. People with PPH may also have a racing pulse or feel that they can’t get enough air. Palpitations (unusually strong or odd heartbeats) can also occur.

How Is Pulmonary Hypertension Diagnosed?

The health care provider first rules out other causes of pulmonary hypertension. These causes include emphysema, pulmonary emboli, heart valve disease, neurologic muscle disorders, and obstructive sleep disorder. Blood tests, X-rays, echocardiography, pulmonary function tests, and imaging studies of the lungs are usually done. Imaging may include nuclear scans, computed tomography (CT), and magnetic resonance imaging (MRI). Cardiac catheterization may also be done if the diagnosis remains unclear. In this test, a tube (catheter) is put into a leg or neck artery and passed into the heart to measure pulmonary artery pressures.

How Is Pulmonary Hypertension Treated?

The main goal is to lower pulmonary artery pressures and increase oxygen levels. Oxygen is given to improve the oxygen level. Strenuous exertion or exercises, which lead to lower oxygen levels in the blood and symptoms, should be avoided. Medicines used in the treatment of pulmonary hypertension include prostanoids (epoprostenol, treprostinil, iloprost, and beraprost). Other effective medicines are endothelin receptor antagonists, phosphodiesterase inhibitors, blood thinners, and calcium-channel blockers. Diuretics (water pills) can be given for ankle and leg swelling. If these treatments don’t help shortness of breath at rest, lung and heart-lung transplantation may be an option.

DOs and DON’Ts in Managing Pulmonary Hypertension:

  • DO understand that PPH has no known cure. However, treatments can improve quality of life.
  • DO see lung and heart specialists who are experienced in the treatment of pulmonary hypertension.
  • DO call your health care provider if your shortness of breath is worse. Call if you have leg swelling, chest pain, or palpitations. Call if you’re thinking of becoming pregnant. Getting pregnant may not be advisable if you have pulmonary hypertension.
  • DON’T be afraid to ask for a second opinion.
  • DON’T smoke!
  • DON’T use birth control pills. They may increase your risk of blood clots.

Contact the following source:

  • National Heart, Lung, and Blood Institute Information Center
    Phone: 301-592-8573
  • Pulmonary Hypertension Association (PHA)
    Phone: 1(800) 748-7274
  • American Lung Association
    Phone: 800-LUNG-USA
  • Centers for Disease Control and Prevention
    Website: fact_sheets/fs_pulmonary_hypertension.htm

Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.

Ferri’s Netter Patient Advisor