BACKGROUND: Cardiovascular disease is the main cause of morbidity and mortality 
in patients with Marfan syndrome. Many patients with presumed Marfan syndrome do 
not meet current diagnostic criteria. This study reviews the surgical aspects of 
aortic disease in 300 patients referred with the diagnosis of Marfan syndrome.
METHODS: During a 16-year period, 300 patients with presumed Marfan syndrome 
underwent 398 operations on the aorta and branch arteries, including 125 aortic 
root operations, 59 aortic arch repairs, 31 descending thoracic aortic repairs, 
and 178 thoracoabdominal aortic repairs. Based on medical record review, 
patients were classified as confirmed Marfan syndrome if documented features 
satisfied current diagnostic criteria; patients not meeting these criteria were 
classified as suspected Marfan syndrome.
RESULTS: There were 17 operative deaths (4.3%) after the 398 operations. 
Survival after the initial referral operation was 96.2% +/- 1.5% at 1 year, 
82.7% +/- 2.4% at 5 years, and 74.6% +/- 3.1% at 10 years. Presentations, 
operative details, and outcomes were remarkably similar in the 137 patients 
(45.7%) with confirmed Marfan syndrome and the 163 patients (54.3%) with 
suspected Marfan syndrome. Freedom from repair failure, however, was 
significantly better in patients with confirmed Marfan syndrome (90.3% +/- 2.3% 
at 10 years) than in those with suspected Marfan syndrome (82.0% +/- 3.1% at 10 
years; p = 0.001).
CONCLUSIONS: Operative treatment of the full spectrum of aortic disease in 
Marfan patients enables excellent long-term survival. Similarities in surgical 
aspects of aortic disease suggest that patients with features of Marfan syndrome 
who do not meet diagnostic criteria should be managed in the same manner as 
patients with confirmed Marfan syndrome.