Risk of Marfan complication could be cut

April 07, 2006|By JONATHAN BOR | JONATHAN BOR,SUN REPORTER

A half-century after a Johns Hopkins physician first described symptoms of Marfan syndrome, researchers there are planning a clinical trial of a drug that prevented a fatal complication in mice.

The condition, which strikes more than 30,000 Americans, weakens the body's connective tissues, making victims unusually tall and lanky but also prone to fatal ruptures of the aorta, the artery that carries blood exiting the heart.

Today's edition of the journal Science carries hopeful news that a common hypertension drug prevented aortic weakening in mice and restored the aortas to their normal size. The researchers plan to begin testing the drug, losartan, next fall among an estimated 1,000 people.

Dr. Harry Dietz, the senior author, said the drug appears to work by blocking a growth factor that causes the aortic wall to thin. The same growth factor is implicated in more common forms of aortic aneurysms, Dietz said, and further studies could determine whether the drug is effective against those.

"We're hoping that the foundation we get from studying Marfan syndrome will provide insight into the more common presentations of aortic aneurysms," Dietz said yesterday.

To date, the only effective remedy for weakened aortas is a procedure in which surgeons repair the artery with Dacron grafts and artificial valves.

"It's estimated that the majority, maybe 90 percent, will require cardiovascular surgery at some point or otherwise die of an aortic tear," Dietz said.

Dr. Vincent Gott, a Hopkins surgeon who has repaired the aortas of more than 100 Marfan patients, said the study provides hope that medicine could make surgery unnecessary.

In 1955, Dr. Victor McKusick, a Hopkins physician who is considered the father of medical genetics, diagnosed Marfan in a young man who had been admitted to the hospital with a serious malfunction of his aorta.

In 1991, Dietz led a team that identified the condition's genetic underpinnings and created a strain of Marfan-afflicted mice that could be used for research.

Patients with aortic complications often take anti-hypertensive drugs known as beta blockers that slow but don't stop the aorta fromweakening, doctors say.

People with Marfan syndrome also commonly suffer from vision problems caused by displacement of the eye lens, as well as orthodontic problems that result from an abnormally narrow jaw and high-arched palate.

The disease gained public attention in 1986 when Flo Hyman, a three-time All-American volleyball player, collapsed and died of a ruptured aorta during a match in Japan. Among its other victims was Chris Patton, a University of Maryland basketball player who died during a pickup game in 1976.

jonathan.bor@baltsun.com

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