Miscellaneous
Life expectancy in the Marfan syndrome

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Abstract

Data reported in 1972 indicated that lifespan in patients with the Marfan syndrome is markedly shortened, and that most deaths are cardiovascular. This study was performed to determine whether survival in the Marfan syndrome has changed since 1972, and to discern whether treatment (medical or surgical) has altered prognosis. Survival curves were generated on 417 patients from 4 referral centers, with a definite diagnosis of the Marfan syndrome. Birth date, age at death, cardiovascular surgery, or treatment with β blockers, or any combination of these, were included in the analysis. Forty-seven of 417 patients died. Mean age at death (41 ± 18 years) was significantly increased compared with age in 1972 (32 ± 16 years, p = 0.0023). Median (50%) cumulative probability of survival in 1993 was 72 years compared with 48 years in 1972. Of 112 surgically treated patients, 10-year probability of survival was 70%. Patients undergoing surgery after 1980 enjoyed significantly increased survival than patients who had undergone operation before 1980 (p = 0.008).

In conclusion, life expectancy for patients with the Marfan syndrome has increased >25% since 1972. Reasons for this dramatic increase may include (1) an overall improvement in population life expectancy, (2) benefits arising from cardiovascular surgery, and (3) greater proportion of milder cases due to increased frequency of diagnosis. Medical therapy (including β blockers) was also associated with an increase in probable survival.

References (18)

  • RomanMJ et al.

    The prognostic significance of the pattern of aortic root dilatation in the Marfan syndrome

    J Am Coll Cardiol

    (1993)
  • CutlerS et al.

    Maximum utilization of the lifetable method in analyzing survival

    J Chron Dis

    (1958)
  • PyeritzRE et al.

    The Marfan syndrome: diagnosis and management

    N Engl J Med

    (1979)
  • MurdochJL et al.

    Life expectancy and causes of death in the Marfan syndrome

    N Engl J Med

    (1972)
  • CrawfordES

    Marfan's syndrome. Broad spectral surgical treatment of cardiovascular manifestations

    Ann Surg

    (1983)
  • KouchoukosNT et al.

    Sixteen-year experience with aortic root replacement. Results of 172 operations

    Ann Surg

    (1991)
  • GottVL et al.

    Composite graft repair of Marfan aneurysm of the ascending aorta: results in 100 patients

    Ann Thorac Surg

    (1991)
  • ShoresJ et al.

    Progression of aortic dilation and the benefit of long-term β-adrenergic blockade in Marfan's syndrome

    N Engl J Med

    (1994)
  • BeightonP et al.

    International nosology of heritable disorders of connective tissue, Berlin 1986

    Am J Med Genet

    (1988)
There are more references available in the full text version of this article.

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This study was supported in part by Grants-in-Aid from the American Heart Association, Dallas, Texas; the Coles Family Foundation, New York, New York; the North Atlantic Treaty Organization, Brussels, Belgium; and the National Marfan Foundation, Port Washington, New York.

1

Dr. Silverman is the recipient of a Young Investigator Award from the Patrick and Catherine Weldon Donaghue Foundation.

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