Family history of severe cardiovascular disease in Marfan syndrome is associated with increased aortic diameter and decreased survival.
OBJECTIVE
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.
Silverman
D I
DI
Division of Cardiology, University of Connecticut Health Center, Farmington 06030, USA.
Gray
J
J
Roman
M J
MJ
Bridges
A
A
Burton
K
K
Boxer
M
M
Devereux
R B
RB
Tsipouras
P
P
D000328:Adult; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging*; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging*; D002318:Cardiovascular Diseases / Q000000981:diagnostic imaging / Q000235:genetics* / Q000401:mortality; D016022:Case-Control Studies; D004108:Dilatation, Pathologic / Q000000981:diagnostic imaging; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D015993:Life Tables; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality*; D010375:Pedigree; D011379:Prognosis; D016016:Proportional Hazards Models; D012307:Risk Factors; D016019:Survival Analysis