Diagnosing and Treating Pulmonary Arterial Hypertension
The treatment for PAH is very complicated and depends on many factors.
What to Expect
The diagnosis of PAH requires numerous tests to determine the type and severity of pulmonary hypertension that you have and treatment is complicated and depends on many factors.
If PAH is suspected, you should expect several tests such as blood tests, breathing tests, X-rays, and possibly procedures to directly measure the blood pressure in the lungs.
How Pulmonary Arterial Hypertension Is Diagnosed
The real key to diagnosis is suspicion of the problem. Diagnosis often requires the help of both a lung and heart specialist (pulmonologist and cardiologist). Patients often have the disease for a while before it is diagnosed. Tests commonly used to diagnose all forms of pulmonary hypertension, including PAH, are:
- Blood tests: Include HIV, thyroid tests, autoimmune disease panels (test for systemic lupus erythematosus, scleroderma, and rheumatoid arthritis), liver tests, and blood chemistry tests.
- Electrocardiogram: Shows the electrical activity of the heart, and may help detect abnormalities. Doctors may also be able to detect signs of strain on the right side of the heart.
- Breathing tests (pulmonary function tests): Check for diseases like asthma or emphysema
- 6 minute walk test: Objectively measures how far you can walk and to see if your oxygen levels drop when you exert yourself.
- Echocardiogram: Ultrasound of the heart to check the size and condition of the chambers of the heart. It can also be used to estimate the blood pressure in the pulmonary arteries.
- Right Heart Catheterization: Because this test is invasive, it is not usually performed unless other tests cannot produce a firm diagnosis. It involves inserting a catheter (small tube) into a large vein in either the neck, arm, or groin, and threading it through the right side of the heart and into the pulmonary artery. This allows measurement of the blood pressure in the lungs.
How Pulmonary Arterial Hypertension Is Treated
The treatment of PAH has change rapidly over the last 15 years. PAH-specific medications come in pill, inhaled, and intravenous (IV)/subcutaneous forms. The medications are often used in a variety of combinations. The treatment of pulmonary arterial hypertension is very dependent on the patient, the severity of the symptoms, the test findings, and even the support that the patient has at home. In addition to specific drugs, patients with PAH are also often treated with anticoagulants (blood thinners) and supplemental oxygen either at night, as needed or continuously. There are also patients with PAH who would benefit from a specific program of pulmonary rehabilitation. All of these potential options should be discussed between the patient and the treating physician. If the disease continues to progress in spite of treatment, your doctor may talk to you about lung transplantation.
Approved by Scientific and Medical Editorial Review Panel. Last reviewed August 4, 2016.