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.