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J Am Coll Cardiol. 1995 Oct;26(4):1062-7.

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

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1
Division of Cardiology, University of Connecticut Health Center, Farmington 06030, USA.

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 (chi-square 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.

PMID:
7560600
DOI:
10.1016/0735-1097(95)00258-0
[Indexed for MEDLINE]
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