May 7, 2012 12:06 am by Pohla Smith / Pittsburgh Post-Gazette
About a year ago, Jean Magazzu was diagnosed with bronchitis, but after two courses of different antibiotics, her symptoms remained. by June, she had extreme shortness of breath.
“I couldn't get up and walk across the room without having to rest,” said Ms. Magazzu, 44, of Mt. Lebanon. a single mother and the office and development coordinator for Outreach Teen and Family Services, she went to St. Clair Hospital's emergency room, where a scan showed she had blood clots in her lungs.
After spending the night at St. Clair, she was transferred to Allegheny General Hospital, where she was immediately put on blood-thinning medication. During her stay of about a week, doctors conducted tests that found that the blood pressure in her lungs was three to four times above normal; the right side of her heart also was enlarged. They placed a filter in the large vein that brings de-oxygenated blood from the lower part of her body into her heart's right atrium to prevent any more clots from reaching her lungs.
Ms. Magazzu had a rare disease called chronic thromboembolic pulmonary hypertension, better known as CTEPH. It's a condition that eventually would have killed her if she had not undergone a complex surgery at Allegheny General on Feb. 9.
This delicate surgery called pulmonary thromboendarterectomy removes the clots from the pulmonary arteries and restores blood flow. it is done at about 10 medical centers across the United States, and Ms. Magazzu was the first patient to undergo the procedure at Allegheny General. to become proficient, a team led by cardiothoracic surgeon Robert Moraca underwent six months of training under David McGiffin at the University of Alabama at Birmingham. Dr. McGiffin was present for Ms. Magazzu's operation.
Before Ms. Magazzu's case, Dr. Moraca said, Allegheny General sent such patients to Birmingham, one of the two best-known centers for the surgery in the nation. The other is the University of California, San Diego, where the procedure was developed.
The condition is caused when a clot breaks off in the legs (or, sometimes, the upper extremities), travels through the circulatory system, on through the right side of the heart and gets lodged in the lungs. If the body doesn't dissolve it, more can form on top of it, causing a network of scar tissue. “So it propagates itself,” said AGH cardiologist Raymond Benza.
“The body's response to that is to try to seal off that artery so no more clots can form there, so the vessel closes down,” he said. The result is not unlike a plugged-up drain, creating a tremendous back pressure in the right heart,” he said.
As the pressure builds, the right heart tries to keep up by getting larger and larger. “When it does, it loses its elasticity” and its ability to pump blood to the lungs. “People are dying … from a type of heart failure,” Dr. Benza said.
Most times patients and their doctors do not know clots are traveling to the lungs, so it goes undiagnosed. “It's only recognized when people start to develop early symptoms of heart failure — swelling in their ankles, edema in their bellies, fatigue, shortness of breath, and then, when the disease becomes more prominent, they start passing out when they try to do things,” he said. “That's because no circulation is going beyond these clots.”
The first line of treatment is to try to dissolve the clots with anticoagulants. If that doesn't work, surgery follows. without surgery, Dr. Benza said, “it's uniformly fatal,” with patients living less than a year once heart failure becomes prominent.
Drugs are being developed to help those with inoperable disease that may prolong survival, he said, but they are not a substitute for the surgery in those who qualify for it.
The surgery involves opening the chest and can take from four to 10 hours. The patient is put on a heart-lung machine, and the body is cooled down to about 68 degrees. “The reason we do that is we need to shut blood supply off to the body and lungs and open up the right and left pulmonary arteries and begin to take off their scar tissue,” Dr. Moraca said.
“We work very quickly and efficiently to remove the scar tissue, so we do it all at once in sort of one long block,” he said.
Once it's removed, doctors restart the heart, re-warm the patient and close up the surgical area.
Ms. Magazzu, who has a congenital condition that causes the blood clots, said she could tell her breathing had improved within a couple days, although she was still exhausted from the surgery.
She went back to work part time the last week of March and two weeks later reported feeling very well.
“My breathing is great,” she said. “My stamina could use a little improvement — I haven't been real active over the last nine months. … I can walk up a flight of steps now without having to stop.”
The survival rate can be more than 95 percent in patients who have the surgery done at experienced centers, Dr. Benza said.
To prevent further problems, Ms. Magazzu will have to take anticoagulants for life and keep a filter in the vein leading to her heart.
Her prognosis is “to live a normal life expectancy for a young woman,” Dr. Benza said. “Her heart is healed. her right ventricle is normal now, and she's going to have an amazing prognosis.”