Family history of severe cardiovascular disease in Marfan syndrome is associated with increased aortic diameter and decreased survival

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Abstract

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. © 1995 American College of Cardiology.

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Silverman, D. I., Gray, J., Roman, M. J., Bridges, A., Burton, K., Boxer, M., … Tsipouras, P. (1995). Family history of severe cardiovascular disease in Marfan syndrome is associated with increased aortic diameter and decreased survival. Journal of the American College of Cardiology, 26(4), 1062–1067. https://doi.org/10.1016/0735-1097(95)00258-0

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