School of Medicine

Wayne State University School of Medicine

Patient Education

PAH ribbonPulmonary hypertension (PH) represents a group of conditions in which the pressure within the blood vessels of the lungs is increased. This leads to stress and strain of the right side of the heart, which may ultimately cause right-sided heart failure.

PH is sometimes confused with “systemic hypertension” which means high blood pressure in the systemic blood vessels, which affects “left” side of the heart mainly.

In the last decade or so there have been advances in the management of PH resulting in improved symptoms, better quality of life as well as increased survival. At present there are six FDA approved medications to treat this disease.

Unfortunately the symptoms of PH are non-specific and the diagnosis may be delayed in the absence of an appropriate work up. One of the reasons for difficulty in managing this disease is late referral to a specialist.

PH may be Idiopathic (without a known cause) or familial (which runs in families). This used to be previously called “Primary Pulmonary Hypertension”.  Pulmonary hypertension may be associated with other conditions like systemic sclerosis (scleroderma), Lupus and other connective tissue disorders, liver disease, HIV/AIDS, use of weight reducing medications, sickle cell disease and various other conditions.

Other conditions that may cause PH include lung problems like emphysema and chronic bronchitis, pulmonary fibrosis, blood clots in the lungs, Sarcoidosis as well as left sided heart disease. Previously all these conditions were called as “secondary pulmonary hypertension”.

Symptoms of pulmonary hypertension include easy fatigability, shortness of breath, chest pain, swelling of  the feet and belly. In advanced cases patients may pass out.

Patients with pulmonary hypertension need comprehensive work up to confirm the diagnoses as well as to find out the underlying cause. Initially echocardiogram is the best tool for screening but to confirm the diagnosis the patients needs to have a right heart catheterization, which actually measures the pressure in the heart and blood vessels by introducing a catheter into the body. Physicians follow specific procedures and protocols to ascertain essential information, which may include possible response to medications, which helps to determine the best possible therapy.

In addition to a chest x-ray, electrocardiograph (EKG), and breathing test (Pulmonary Function Test), patients may need a CT scan of the chest or a VQ scan to look for other lung diseases or clots in the lungs.

Another important test is a six-minute walk test. This gives the physician an idea about the patient’s capacity to tolerate day-to-day activities, degree of disability and need for oxygen therapy. In addition this test is done periodically to monitor for improvement or worsening and assess the response to medication. Physicians may add or change therapy based on these results.

At present mediations approved for treatment of Pulmonary Arterial Hypertension (PAH) include calcium channel blockers, Flolan (epoprostenol), Remodulin (treprostenol), Ventavis ( iloprost), Tracleer (bosentan), Letairis (ambrisentan) and Revatio (sildenafil). The choice of medication is based on the patient’s condition at the time of diagnosis. Advanced cases may be referred for lung transplant.

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