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Study finds aortic valve replacement benefits Marfan's sufferers
Thursday, April 29, 1999 By Byron Spice, Science Editor, Post-Gazette
Mary Kerchner's mother died at age 40. An aunt died at 15. Several uncles died by their early 20s. A couple of uncles lived the longest -- until their mid-40s. But all seven siblings had weak aortas and all died when that main blood vessel burst suddenly, catastrophically.
Kerchner and her brothers suffered from the same problem -- a connective tissue disorder called Marfan's syndrome. Knowing her time was limited, the York native lived a life that was admittedly wild, perhaps unwise, but always full. Then, at age 30, she underwent a then-experimental operation to replace the weakened portion of her aorta and her aortic valve.
Now, at an age when some women fib about theirs, Kerchner, of Baldwin, is only too happy to proclaim her longevity. "I'm still here at 51," she said yesterday, "which is older than anyone in my family ever got."
Kerchner's operation at Johns Hopkins University in Baltimore replaced her heart's defective aortic valve with an artificial valve and replaced the first few inches of her aorta with a Teflon sleeve. It was considered risky at the time, but an article being published today in the New England Journal of Medicine says it not only is safe, but should be done long before symptoms appear.
As recently as 1972, people with Marfan's had a life expectancy of about 45 years, but this surgery and other treatments have helped extend median survival to age 72.
In the new study, Hopkins Dr. Vincent Gott and his colleagues noted that of 455 patients who had the surgery electively, just 1.5 percent died within 30 days. By comparison, the 117 patients like Kerchner who underwent urgent repair had a 2.5 percent death rate and those who had emergency surgery after the aorta ruptured had a 30-day mortality rate of almost 12 percent.
Dr. Reed Pyeritz, Kerchner's doctor at Hopkins and a co-author of today's article, said the report may convince more surgeons to offer the surgery electively. Some remain hesitant because of fears that patients with Marfan's syndrome face greater surgical risks because of their fragile vessels.
Often associated with Abraham Lincoln, Marfan's syndrome is an inherited condition affecting 200,000 Americans. It can result in unusually long tubular bones, an arm span exceeding height and long, spidery fingers. Kerchner said her family fit this profile; at 6 feet, 2 inches tall, she was dwarfed by the rest of her family, including a 6-11 brother.
The underlying disorder is a defect in a gene that makes the body's connective tissue, the fibrous material that supports the body's organs and maintains elasticity in blood vessels. Eye and joint problems can occur, but weakness in the aorta is the most serious complication. The weakened vessel wall can balloon out, forming an aneurysm that can rupture.
In 1986, U.S. Olympic volleyball player Flo Hyman died when her aorta suddenly burst; 10 years earlier, University of Maryland basketball star Chris Patton died when his aorta exploded during a pickup game. Both had undiagnosed cases of Marfan's syndrome.
Replacing the weakened section of aorta and the weakened aortic valve is one of the bigger open heart operations and, when it originated in the late 1960s and early '70s, no one was sure of the risks, said Pyeritz, now director of human genetics at Allegheny General Hospital. Consequently, many surgeons hesitated to do the surgery unless the patient was suffering symptoms.
Kerchner's aorta was already near bursting by the time she was referred to Hopkins for testing in 1978, Pyeritz recalled. She had been suffering palpitations that kept her awake at night and her heart was greatly enlarged from pumping against a defective aortic valve.
"They told me there was a 75 percent chance I'd make it off the [operating] table," Kerchner recalled. "I had some trepidations, but I knew what was the ultimate outcome" of not having the surgery.
Pyeritz said the surgical technique has been further refined since then. Surgeons now often try to surgically strengthen the aortic valve, rather than inserting an artificial valve, and replace only the weak section of aorta.
In an editorial accompanying today's study, Drs. Richard Devereux and Mary Roman of Cornell University said the surgery has been so successful that it might also be used to extend the lives of people with other kinds of aortic disease.
Kerchner is chairwoman of the Western Pennsylvania support group for Marfan's syndrome. For information, call her at 412-653-4740.
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