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Primary pulmonary hypertension is now known as pulmonary arterial hypertension (PAH). The name was changed in 1998 because it was determined to be more descriptive of the condition.
PAH is a disorder characterized by an abnormal increase in pulmonary artery pressure, normal pulmonary capillary wedge pressure, and increased pulmonary vascular resistance. A gradual increase of pulmonary artery pressure can lead to eventual right-heart failure and death. Increases in pulmonary arterial pressure are the most important indicators of PAH.
Imaging tests are used to look inside the body for a diagnosis. Enlarged arteries or damage to the heart are sure signs of PAH. Medications are used to slow heart damage and deal with symptoms.
Even with treatment, about one-third of people with PAH will die within three years of diagnosis. Thanks to new treatments, this number is an improvement from the 50 percent who used to die within 3 years. The medical community hopes to find a cure to stop the damage of PAH.
The cause of PAH can be either idiopathic (IPAH) or familial (FPAH). Idiopathic diseases arise spontaneously, and their causes are unknown. Familial diseases are genetically passed down through families.
Environmental factors and medications are believed to be possible causes of IPAH.
Possible causes of IPAH:
Most cases of FPAH arise from a mutation in the BMPR2 gene. Severe cases are generally linked to the presence of the mutation. Although the mutation may be present at birth, the disease may take months or years to appear.
The characteristic symptom of PAH is an increase in pulmonary vascular resistance. This causes an increase in pressure on the right ventricle of the heart. That problem leads to trouble in filling the left part of the heart. Eventually, the heart fails as a result.
Common symptoms of PAH are:
PAH is still not well understood. Research has found certain diseases that may make a person more likely to develop PAH:
Diagnosis isn’t easy, because damage inside the heart can’t be seen during a routine examination. Doctors try to rule out all other causes of pulmonary hypertension before a diagnosis is made. Your doctor may order some or all of the following tests:
Treatments for PAH are meant to reduce symptoms and prolong life. Doctors often begin treatments late in the disease, because it’s hard to detect.
The FDA has approved several medications for PAH. They can ease symptoms and improve physical function. None of the medications can completely relieve the symptoms of PAH and some are extremely expensive, costing upward of $175,000 a year.
Although modern treatment has come a long way, one-third of all people with PAH will die within three years of diagnosis.
Death is an expectation and a common occurrence with PAH, because no cure or effective treatment exists. The medications that are available to treat symptoms can extend life for a while, but they can’t stop eventual terminal heart failure.
Written by: Lydia Krause
Medically reviewed on: Mar 15, 2017: Graham Rogers, MD
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