Pulmonary arterial hypertension (PAH) affects proper blood flow through small- and medium-sized blood vessels in the lungs. PAH can cause the right side of the heart to function less efficiently, and over time may lead to heart failure.
Our PAH team includes nurse practitioners, nurse coordinators, pharmacists and pulmonary medicine specialty physicians who work with you to develop a personalized treatment plan that is designed to help you achieve the results you need.
Shortness of breath, the most common symptom, is first noticeable during physical exertion and then as the disease advances. Other symptoms include chest pain, fainting, lower extremity (legs and feet) swelling and heart palpitations.
While there are no clear reasons for the development of PAH, we do know it occurs more often in women ages 20 to 50 than in men. In some cases, a gene mutation has been the cause, and research has shown that those diagnosed with PAH undergo various chemical changes within the body.
Types of PAH
Treatment planning for PAH relies on knowing the type of PAH in relation to the underlying cause or associated disease. The World Health Organization has classified PAH in 5 different groups:
- I PAH includes right heart disease.
- II PAH includes left heart disease or heart valve disease.
- III PAH includes lung diseases and lack of oxygen such as emphysema, pulmonary fibrosis or a spine condition called kyphoscoliosis.
- IV PAH includes chronic blood clots in the lungs (pulmonary embolism).
- V PAH includes various different causes not listed in these other categories.
Each PAH group involves different treatments. Often, treatment of the underlying cause of PAH is the best course of action. Our experienced team will work with you to determine the best methods to treat your PAH.
Diagnostic tests for PAH include:
- Heart studies: An abnormal cardiac ultrasound or two-dimensional echocardiogram can lead to a diagnosis of PAH. These initial tests will prompt the physician to order various other tests to confirm the diagnosis.
- Radiology and pulmonary studies: The array of tests to confirm PAH may include a chest X-ray, pulmonary ventilation/perfusion scans (called V/Q scans), laboratory blood tests, pulmonary function tests or a computed tomography of the chest.
- Right heart catheterization: Most patients require a right heart catheterization to confirm the diagnosis of PAH, which includes inserting a catheter into the right side of the heart and into the pulmonary artery.
At the Henry Ford Pulmonary Hypertension Clinic, frequent visits are required for patients, especially in the beginning of care. Although the visit schedule depends on your unique type of treatment, most patients are seen once every three months. At each follow-up visit, most patients will be given the six-minute walk test as well as other tests, such as blood markers, to help monitor treatment progress. Two-dimensional echocardiograms and right heart catheterizations may also be needed over time.
- Medications: Several medications have been approved for the treatment of PAH. Some medications must be taken in the inhaled form, some injected through the vein (intravenous) or beneath the skin (subcutaneous), and others by mouth. Research has demonstrated that use of certain medications for PAH improves the ability to walk and length of life.
- Surgery: Surgical options for the treatment of PAH in select cases may include lung transplantation or atrial septostomy. Surgery is considered on a case by case basis.
- Supportive Therapies: Supportive therapies for PAH patients include restriction of water intake, use of water pills (diuretics), monitored exercise with pulmonary rehabilitation, and use of certain medications such as digoxin and blood thinners (anticoagulants). The most important goal is to improve your quality of daily life and to detect and prevent health deterioration.
We make the best effort to educate our patients at every visit or to schedule special educational visits for our patients and their families. Pulmonary rehabilitation classes are available to our patients. We also participate in educating patients by attending support groups led by the Pulmonary Hypertension Association, Detroit Chapter.