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New Study Reveals Dramatic Increase in Life Expectancy for People With Marfan Syndrome
FARMINGTON, CT, January 17, 1995 ? The life expectancy for patients with Marfan Syndrome, a potentially fatal genetic disorder of the connective tissue, has increased more than 25 percent since 1972, according to research published in the January 15, 1995, issue of the American Journal of Cardiology. In fact, the study reports that the median cumulative probability of survival (the age at which 50% of patients are predicted to be alive) has risen from 49 to 74 years for women and from 41 to 70 years for men in the past 32 years.
"Our data indicate that median survival for patients with the Marfan Syndrome has increased significantly compared with the only series previously reported in 1972," says Petros Tsipouras, M.D., University of Connecticut Health Center, who participated in the research. "To the extent that our study population more fully represents patients with clinically mild forms of the Marfan Syndrome, it provides survival information directly applicable to the entire current spectrum of patients with the disorder."
"These findings provide great hope for people who have Marfan Syndrome and those who have yet to be diagnosed," says Priscilla Ciccariello, chair, National Marfan Foundation. "However, research challenges do remain because there is still no single routine laboratory test to diagnose the disorder and there is no cure."
The Marfan Syndrome
The Marfan Syndrome and related connective tissue disorders affect at least 200,000 people in the United States. Because connective tissue is the glue and scaffolding of the entire body, the disorder manifests itself in many ways, including the bones and ligaments, eyes, heart and blood vessels. It is the effect on the aorta, the largest blood vessel carrying blood away from the heart, that can be fatal. Many people with Marfan Syndrome experience an expansion of the aorta; without proper monitoring and medications to reduce the stress on the aorta, it could tear, resulting in sudden death. This was the case of U.S. Olympic volleyball star Flo Hyman, who died of a ruptured aorta in 1986; she did not even know she had Marfan Syndrome.
Researchers studied data from 417 patients from Marfan specialty clinics at the University of Connecticut, Farmington; Ninewells Hospital, Scotland; Jewish Hospital, St. Louis; and New York Hospital/Cornell Medical Center, New York. All patients had a definitive diagnosis of Marfan Syndrome. Mean follow-up (time from the first presentation in the clinic to the present date or death) was 5.2 ± 3.6 years.
According to the study, reasons for the increased life expectancy may include:
Advanced cardiovascular surgery, both prophylactic and emergent, which has become accepted treatment for aortic and mitral regurgitation, aortic aneurysm and aortic dissection. Increased options in medical therapy, including-adrenergic receptor antagonists, which have gained wide acceptance as potential agents for delaying aortic expansion and progression to rupture or dissection. Greater proportion of milder cases due to increased frequency of diagnosis. Overall improvement in the life expectancy of the general population.
In addition to Dr. Tsipouras, the following investigators participated in the research: David I. Silverman, M.D., Kevin J. Burton, B.S., Jonathon Gray, M.D., Matthew S. Bosner, M.D., Nicholas T. Kouchoukos, M.D., Mary J. Roman, M.D., Maureen Boxer, Ph.D., and Richard Devereux, M.D.
Additional findings of note include the following:
For boys and girls in the study population who reached the age of 10, the probability of surviving to the age of 20 was nearly the same in 1994 as in 1972. However, by the age of 20 years, the difference in the probability of surviving to the age of 40 hadincreased dramatically for the study population for both men and women. This gap continued to widen through middle age.
For the surgical group of the study population (27%), the cumulative probability of survival demonstrates a five-year survival rate of 82 percent and a ten-year survival rate of 70 percent. Of patients who underwent surgery prior to 1980, 63 percent have died, compared with only 16 percent of patients who underwent surgery after 1980.
Median cumulative probability of survival for patients who had taken beta-blockers was 72 years, compared with 70 years for patients who had never taken beta-blockers. Mean age at surgery was 33 ± 11 years for patients who took beta-blockers, compared with 29 ± 10 years for those who did not.
For additional information about Marfan Syndrome, contact the National Marfan Foundation toll-free, 800-8-MARFAN or write to the NMF at 22 Manhasset Avenuet, Port Washington, NY 11050.