BACKGROUND: The diagnosis and surgical treatment of patients with Marfan 
syndrome remain controversial. It is of utmost importance to identify patients 
at risk for acute aortic events to establish the correct surgical timing and the 
appropriate surgical treatment.
METHODS: From May 2008 to December 2012, 500 patients were screened at the 
Marfan Presidium of the Tor Vergata University Hospital of Rome (Italy). 
Patients were evaluated by a cardiac surgeon, including echocardiographic, 
orthopedic, ophthalmologic and dental examinations. All patients received 
genetic counseling, and genetic sampling was performed if appropriate.
RESULTS: The diagnosis of Marfan syndrome was confirmed in 146 patients (29.2%). 
Fifty-four patients (37%) underwent cardiac surgery on the aortic root, 4 
patients had surgery on the mitral valve, 13 patients had combined surgery; 11 
cases were emergent surgery for acute aortic dissection. Twenty-eight patients 
(52%) were operated on at our Division: 13 underwent valve-sparing aortic root 
replacement (David procedure), 1 underwent Yacoub remodeling procedure and 14 
underwent Bentall procedure. Following the establishment of the Marfan Center, 
the David aortic valve-sparing operation was the most frequently performed 
procedure compared to the previous period of surgical activity (63 vs 22%, 
p<0.0001).
CONCLUSIONS: Regular follow-up twice a year may allow to identify patients at 
risk for acute aortic syndromes. Early surgical treatment is recommended in 
these patients to achieve optimal results of valve-sparing procedures and 
life-saving management, especially for patients who live far away from a cardiac 
surgery center.