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What Is Pulmonary Arterial Hypertension?

Jan 11, 2016

Singer Natalie Cole was laid to rest today in Los Angeles, less than a week after dying of heart failure caused by pulmonary arterial hypertension.

"Pulmonary Arterial Hypertension or PAH is a rare condition where the blood pressure in the lungs increases," says John Ryan, MD, a cardiovascular specialist with University of Utah Health Care. "This puts a lot of strain on the heart and ultimately can result in heart failure and death."

Cole was first diagnosed with PAH in 2009 following a kidney transplant. Doctors have said that the cause of her disease was idiopathic, or unknown. However, there are some behaviors and conditions that can bring on PAH. "Diet pills like Fen-Phen and Aminorex can cause PAH, as can methamphetamine use," says Ryan. "Other causes include auto-immune diseases, such as lupus and scleroderma, HIV, schistosomiasis or rheumatoid arthritis."

Catching PAH early is key when it comes to long-term survival. That's not an easy feat because many of the symptoms can be non-specific and may come on slowly. "The symptoms of PAH may be things that don't point to a problem with the lungs, like leg swelling or abdominal swelling," Ryan says. "They also can include shortness of breath, fatigue, loss of consciousness or chest pain."

The most common treatment for PAH may surprise you. It's Viagra. That's right: A drug most commonly associated with erectile dysfunction is helping PAH patients live full lives.

"Viagra dilates blood vessels — that is how it works in men with ED," says Ryan. "In PAH, medicines such as sildenafil dilate the blood flow to the lungs in an effort to decrease the blood pressure in the lung and increase the amount of blood flow through the lung."

Other recommendations for PAH patients include getting enough sleep, avoiding high altitudes, not smoking and keeping blood pressure from going too low. In more advanced cases of PAH, a lung transplant may be recommended.