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Pulmonary hypertension gets a closer look
Wednesday, October 11, 2006

 
 
 
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To register for this weekend's conference, call 1-866-619-4697.

 
 
 

Six years ago, when she traveled to Pittsburgh from her home in Mexico, Rebeca Dosal found that a medication was available to treat her pulmonary arterial hypertension.

Ms. Dosal, 39, who has had the disorder since birth, had been told before that transplant surgery was the only alternative.

But the new medication and another that was later prescribed eventually got her shortness of breath under control. Today, Ms. Dosal, of Shadyside, is able to take care of her children, ages 8 and 10, and perform volunteer work.

A conference that will be held Downtown this weekend will emphasize that a variety of treatments are available for the often disabling, even life-threatening condition.

"Pulmonary Arterial Hypertension Today and Tomorrow" will be held Saturday at the Omni William Penn Hotel. The free conference includes sessions for health care providers and for patients and their family members.

Similar conferences have been held recently in California, Michigan and North Carolina and others are scheduled next month in Philadelphia and Houston. The meetings are sponsored by the Pulmonary Hypertension Association and the University of Michigan medical school.

Speakers at the Pittsburgh session include Dr. Srinivas Murali, medical director of the Gerald McGinnis Cardiovascular Institute at Allegheny General Hospital, and Dr. Michael Mathier, director of the pulmonary hypertension program at the University of Pittsburgh Medical Center.

While the condition has been considered rare, it is underdiagnosed, said Rino Aldrighetti, president of the Pulmonary Hypertension Association and another speaker at this weekend's conference.

Delay in diagnosis continues to be a problem, Dr. Mathier said, noting that patients often are diagnosed initially with heart problems or asthma.

"There have been a lot of misdiagnoses and a lot of missed diagnoses," Mr. Aldrighetti said.

But doctors are paying closer attention to the illness, in part because "the tools are out there" to treat it, he said.

Some are oral medications, while others require breathing treatments. Still others are administered intravenously or under the skin, Dr. Mathier said.

Pulmonary arterial hypertension, formerly known as primary pulmonary hypertension, is characterized by increased pressure in the pulmonary artery, which carries oxygen-poor blood from the right ventricle of the heart to the lungs. There, the blood is oxygenated and returned to the heart, where it is pumped out to the rest of the body.

The increased pulmonary artery pressure forces the right ventricle to work harder, and the heart can be severely damaged over time.

Symptoms of pulmonary arterial hypertension often include fatigue and shortness of breath.

People with certain health problems that include scleroderma, HIV or liver disease, or those who have taken certain weight-loss drugs, are at higher risk for the condition. It can occur in all age ranges, though it appears to occur more often in women than in men, Dr. Murali said.

For years, lung transplants were the only treatment for primary arterial hypertension, and they remain the only cure. Sometimes, patients with severe heart damage also require heart transplants.

Into the 1990s, the two-year survival rate for people with the condition was about 50-50, Dr. Mathier said. But today, about 85 percent or more of patients survive that long.

Researchers have found that excesses or deficiencies of certain substances within the pulmonary artery are linked to a progression of the disease, and several medications have been developed to address those problems, Dr. Murali said. Research into gene and stem cell therapies is also under way, he said.

While the new medications are often effective in slowing the progress of the disease, some patients still require transplantation.

One of them is David Rocco, 17, of Cranberry, who had a heart transplant and double lung transplant last month.

His mother, Linda, said that at age 13, her son began having trouble keeping up with the ball when he played soccer.

"We thought he just looked out of shape, not realizing he couldn't breathe," she said.

Fainting episodes helped lead to a diagnosis of sports-induced asthma. When treatment for that problem didn't help, doctors eventually diagnosed him with pulmonary arterial hypertension.

"They told us he had three to five years to live without treatment," she said. Initially, he was given a medication that he had to carry around with him and that was infused through a tube in his chest, she said. But despite that treatment and other therapy, he continued to decline.

Mrs. Rocco, whose family has raised $150,000 to battle the disease, said he is recovering well and is looking forward to the cure offered by the surgery.

"We're just very thankful we're in Pittsburgh, where these types of miracles take place," she said.

For a time, Ms. Dosal felt she also might need a transplant.

She said her pulmonary arterial hypertension, which resulted from a congenital heart problem, was diagnosed when she was 10. For years, she experienced few symptoms.

But her symptoms worsened in the 1990s, and she traveled to Pittsburgh from Mexico to be evaluated for a transplant.

Though they improved before she had the surgery, the symptoms later worsened again, prompting her to return to Pittsburgh with her two children in 2000.

By then, an experimental drug was available, and one of her doctors suggested that she enroll in the clinical trial. She was later switched to another medicine and, over time, her symptoms improved. Gradually, she was able to handle an active schedule.

Her message is that it's often possible, with current treatments, to live with the condition.

"It is a life-threatening illness, but you never know," she said. "It's in God's hands."

First published on October 11, 2006 at 12:00 am
Joe Fahy can be reached at jfahy@post-gazette.com or 412-263-1722.
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