EP34 ROLE OF SPECIALIZED CENTRE AND TEAMWORK IN THE DIAGNOSIS OF MARFAN SYNDROME AND PREVENTION OF ACUTE AORTIC DISSECTION

Abstract

Objective: Marfan patients had an increasing risk of aortic dissection related to the quality of the aortic wall. The aortic size is not a safe indicator to prevent aortic dissection. Other factors need to be consedered such as the morphology of the aortic root, the Valsalva sinus prolapse, genetic and clinical aspects. We analysed the role of a specialized centre and a good teamwork in improving Marfan syndrome diagnosis and optimizing surgical indications. Materials and Methods: We enrolled patients admitted to the Marfan Syndrome Centre of Tor Vergata University from March 2008 to December 2017. These patients have been evaluated by a teamwork composed by different figures: cardiologists, cardiac surgeons, oculists, orthopaedics, dentists, genetics and nurses. Results: From 1323 patients admitted, we enrolled a total of 460 patients. Of these 260 patients had Marfan Syndrome, 10 patients had Loeys-Dietz Syndrome and the others 190 were Marfan like. 95 patients underwent cardiac surgery. In particular 47% had a Bentall-DeBono Operation, 40% a David Operation and 13% other operations. An increasing number of surgical procedures has been observed in the years. Accordingly we noticed a reducing incidence of acute aortic dissection and sudden deaths in our patients. Conclusions: A specialized Centre of Rare Disease is very important in the management of Marfan's patients in order to improve the diagnosis, optimize surgical indications and prevent catastrophic complication like acute aortic dissection and rupture.