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

J Am Coll Cardiol. 1995 Oct;26(4):1062-7. doi: 10.1016/0735-1097(95)00258-0.

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Aorta, Abdominal / diagnostic imaging*
  • Aorta, Thoracic / diagnostic imaging*
  • Cardiovascular Diseases / diagnostic imaging
  • Cardiovascular Diseases / genetics*
  • Cardiovascular Diseases / mortality
  • Case-Control Studies
  • Dilatation, Pathologic / diagnostic imaging
  • Echocardiography, Doppler
  • Female
  • Humans
  • Life Tables
  • Male
  • Marfan Syndrome / complications*
  • Marfan Syndrome / mortality*
  • Pedigree
  • Prognosis
  • Proportional Hazards Models
  • Risk Factors
  • Survival Analysis