Data reported in 1972 indicated that lifespan in patients with the Mar
fan syndrome is markedly shortened, and that most deaths are cardiovas
cular. This study was performed to determine whether survival in the M
arfan syndrome has changed since 1972, and to discern whether treatmen
t (medical or surgical) has altered prognosis. Survival curves were ge
nerated on 417 patients from 4 referral centers, with a definite diagn
osis of the Marfan syndrome. Birth date, age at death, cardiovascular
surgery, or treatment with beta 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 w
ith age in 1972 (32 +/- 16 years, p = 0.0023). Median (50%) cumulative
probability of survival in 1993 was 72 years compared with 48 years i
n 1972. Of 112 surgically treated patients, 10-year probability of sur
vival was 70%. Patients undergoing surgery after 1980 enjoyed signific
antly increased survival than patients who had undergone operation bef
ore 1980 (p = 0.008). In conclusion, life expectancy for patients with
the Marfan syndrome has increased Reasons for this dramatic increase
overall improvement in population benefits arising from cardiovascular
surgery, and (3) greater proportion of milder cases due to increased
frequency of diagnosis. Medical therapy (including beta blockers) was
also associated with an increase in probable survival.