Robert Wood Johnson University Hospital

Primary Pulmonary Hypertension

Primary Pulmonary Hypertension

What is primary pulmonary hypertension (PPH)?

Pulmonary hypertension is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels.

Primary pulmonary hypertension has been associated with the appetite suppressants fenfluramine and dexfenfluramine (fen/phen), which were taken off the market in 1997. In the U.S., there are an estimated 500 to 1,000 new cases diagnosed each year, but the actual number of cases is unknown. It is most common in women between the ages of 21 and 40; however, anyone can develop it.

What are the causes of PPH?

The exact cause of PPH is unknown. Research has linked primary pulmonary hypertension to genetic or familial predisposition. Researchers believe the blood vessels are particularly sensitive to certain internal or external factors, and constrict, or narrow, when exposed to these factors, such as an immune system factor, or sensitivity to drugs or other chemicals.

Secondary pulmonary hypertension occurs as a result of the effects of other conditions which may include diseases of the heart or lungs, a blood clot in the lungs, or a condition called scleroderma.

What are the symptoms of PPH?

The following are the most common symptoms for pulmonary hypertension. However, each individual may experience symptoms differently. Symptoms may include:

More severe symptoms indicate a more advanced disease. In advanced stages, the patient:

The symptoms of primary pulmonary hypertension may resemble other conditions or medical problems. Consult your doctor for a diagnosis.

How is PPH diagnosed?

Pulmonary hypertension is rarely discovered in a routine medical examination, and in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.

Pulmonary hypertension is a diagnosis of exclusion. Diagnostic procedures may include:

Treatment for PPH

Specific treatment will be determined by your doctor based on:

Treatment may include one or more of the following:

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