Pulmonary Arterial Hypertension (PAH)

UCLA is one of the largest centers in the country for treating patients with PAH. PAH is defined as elevated blood pressure in the lungs, which leads to enlargement of the right side of the heart and, if untreated, heart failure and death.

Fortunately, as a result of research, including that of UCLA Pulmonary Vascular Disease physicians, there are now numerous effective treatment options for PAH. Determining which option is best for an individual patient and following patients on treatment requires an experienced team of physicians, nurse practitioners and nurses.

Diagnosis of PAH

The diagnosis of PAH requires first recognizing suspicious symptoms. These include:

• Shortness of breath, especially with exertion
• Chest pain
• Fatigue
• Dizziness or passing out
• Leg or abdominal swelling
• Cough

We also ask about risk factors that could make PAH more likely, including:

• An underlying connective tissue (autoimmune) disease such as scleroderma or lupus
• Family history of pulmonary hypertension
• Symptoms suggestive of sleep apnea
• History of liver problems
• History of diet or other stimulant drug use

Once a diagnosis of PAH is considered, several tests may be performed. These include

• Echocardiogram: An ultrasound of your heart looking for elevated pressures in the pulmonary artery and enlargement of the right side of the heart
• Pulmonary function tests: Breathing tests to evaluate your lung function and the ability of the lungs to take in oxygen
• V/Q scan: A nuclear medicine test to evaluate for blockages (such as blood clots) in the pulmonary arteries (as is seen in a condition called CTEPH)
• Right heart catheterization: A procedure in which a catheter is inserted through a large vein in your neck, arm or leg, and advanced into the heart and pulmonary arteries to measure the pressures in the heart and lungs. View more information about right heart catheterizations >

Treatment

We have developed many effective treatments for PAH.  These medications range from once a day pills to infusion pumps that deliver medication continuously through an implanted catheter. Which medications are right for you will depend on a number of factors (severity of your condition, type of PH you have, tolerability in terms of side effects, etc…)  The physicians and nurse practitioners in the UCLA Pulmonary Vascular Programs, all international leaders in the field, have decades of experience in treating PAH and will provide the best, state of the art care to you.  

View a list of PAH medications >

In some cases, lung transplantation may be the best option.  If this is the case, we can make a referral to the UCLA lung transplant program, one of the largest in the country.