We don't know the cause; there may be one or more causes.
The low incidence makes learning more about the disease extremely difficult.
Studies of PPH also have been difficult because a good animal model of the disease hasn't been available.
We think that in most people who develop primary pulmonary hypertension, the blood vessels are very sensitive to certain factors that trigger this disease to develop.
- For example, people with Raynaud's syndrome seem more likely than others to develop PPH.
- In addition, appetite suppressants, cocaine and HIV are some factors believed to trigger the constriction, or narrowing, of the pulmonary artery.
What are the symptoms of primary pulmonary hypertension?
- The first symptom is often fatigue or tiredness. Many patients think that they're simply "out of shape."
- Difficulty in breathing, dizziness and even fainting spells can occur.
- Swelling in the ankles or legs, bluish discoloration of the lips and skin, and chest pain more often occur later in the disease.
How is a patient with primary pulmonary hypertension evaluated?
Significant advances in PPH therapy over the past decade have markedly affected the survival and quality of life for people with this disease.
The optimal medical and/or surgical treatment for patients with PPH depends upon a thorough evaluation at a medical center with expertise in pulmonary hypertension.
The evaluation includes a right heart cardiac catheterization.
In this procedure the doctor places a thin, flexible tube (a catheter) through an artery or vein in the patient's arm, leg or neck, then threads it into the right ventricle and pulmonary artery.
This is the only way to measure the pressure in the pulmonary artery and find out what medical therapy is appropriate for a given patient.
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