Author + information
- Received June 24, 1994
- Revision received April 28, 1995
- Accepted May 5, 1995
- Published online October 1, 1995.
- David I. Silverman, MD, FACCa,*,1,
- Jonathon Gray, MDa,*,
- Mary J. Roman, MD, FACCa,†,
- Allan Bridges, MDa,*,
- Kevin Burton, BSa,
- Maureen Boxer, PhDa,*,
- Richard B. Devereux, MD, FACCa,* and
- Petros Tsipouras, MDa
- ↵*Address for correspondence: Dr. David I. Silverman, Cardiology, L3108. University of Connecticut Health Center, Farmington, Connecticut 06030.
Objectives. We attempted to determine whether a family history of severe cardiovascular disease in patients with the Marfan syndrome is associated with increased aortic dilation or decreased survival, or both.
Background. The prognostic importance of a family history of severe cardiovascular disease in patients with the Marfan syndrome has been incompletely examined. We hypothesized that such a family history would correlate with increased aortic dilation and would be associated with decreased survival.
Methods. One hundred eight affected patients and 48 unaffected family members from 33 multigenerational families with the Marfan syndrome underwent echocardiographic measurement of the aortic root, arch and mid-abdominal aorta. Date of birth and age at death ascertained from family pedigrees were used to perform life table analysis and estimate survival.
Results. Aortic root and arch diameters were significantly greater in patients with a family history of severe cardiovascular disease than in patients without such a family history. Of subjects in the highest quartile for aortic size, >80% had such a family history in contrast to <10% of those in the lowest quartile (chisquare 57.37, p < 0.00001). Mean age at death and cumulative probability of survival were significantly lower in patients with such a family history.
Conclusions. Among patients with the Marfan syndrome, aortic dilation is greater and life expectancy shorter in those with a family history of severe cardiovascular manifestations. These data suggest that such a family history is an important risk factor for cardiovascular events in patients with the Marfan syndrome.
↵1 Dr. Silverman is the recipient of a Young Investigator Award from the Patrick and Catherine Weldon Donaghue Foundation, Hartford, Connecticut.
This study was conducted with support from Grant MO1RR06192 from the General Clinical Research Center and supported in part by a Grant-in-Aid from the American Heart Association, Dallas, Texas (Drs. Tsipouras and Devereux), the Coles Family Foundation (Dr. Tsipouras), the National Marfan Foundation (Dr. Tsipouras) and the North Atlantic Treaty Organization (Dr. Silverman).
- Received June 24, 1994.
- Revision received April 28, 1995.
- Accepted May 5, 1995.
- American College of Cardiology