HERSHEY – Pulmonary arterial hypertension is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels. Left untreated, PAH may become life threatening and often produces severe symptoms like shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting.
When PAH occurs in the absence of a known cause, it is referred to as primary pulmonary hypertension. There are likely many unknown causes of PPH. It is extremely rare, occurring in about two out of every million people in a given year.
A more common variety is secondary pulmonary arterial hypertension, in which the cause is known. Common causes of SPH are emphysema and bronchitis, also called COPD. Congenital heart diseases that cause shunting of extra blood through the lungs, such as ventricular and atrial septal defects, are other causes. Old blood clots in the lung arteries, HIV (the AIDS virus) infection, liver disease and diet drugs like fenfluramine and dexfenfluramine also are causes of SPH.
PAH should be considered when more common causes of similar symptoms have been ruled out. These include heart and lung problems. Most cases of PAH should be found during an echocardiogram, which is an ultrasound of the heart. An “echo” is almost always performed to evaluate breathing problems, but if it isn’t, most doctors will perform one if the patient suggests it.
Unfortunately, an echo may miss PAH and it is frequently misdiagnosed. Often it progresses to a late stage by the time it is accurately diagnosed. The only way to can completely rule out PAH is with a right heart catheterization. In this test, a plastic tube is advanced through the blood stream and right heart into the lung blood vessels. It also may be combined with a left heart catheterization done to examine the heart arteries and open a blockage.
In the past, PAH was thought to be chronic and incurable with a poor survival rate. However, new treatments are available, which have significantly improved prognoses. Recent data indicate that the length of survival is continuing to improve, with some patients able to manage the disorder for 15 to 20 years or longer.
A good source of information is the Pulmonary Hypertension Association, which was founded by patients. It has links to PAH specialists and support groups. The toll-free help line is (800) 748-7274. You can visit Pulmonary Hypertension Association online.