LIFE EXPECTANCY IN THE MARFAN-SYNDROME

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.