title: Surgical management of patients with Marfan syndrome: evolution throughout the years.
abstract: OBJECTIVE
  To evaluate the evolution of surgical management in a large population of patients with Marfan syndrome.
  
  
  METHODS
  This is a retrospective study of patients fulfilling the Ghent criteria for Marfan syndrome, who visited the Centre de référence national pour le syndrome de Marfan et apparentés and underwent a surgical event before or during follow-up in the centre.
  
  
  RESULTS
  One thousand and ninety-seven patients with Marfan syndrome, according to international criteria, came to the clinic between 1996 and 2010. Aortic surgery was performed in 249 patients (22.7%; 20 children and 229 adults), including the Bentall procedure in 140 patients (56%) and valve-sparing surgery in 88 patients (35%); a supracoronary graft was performed in 19 patients (7.6%), usually for aortic dissection. During the past 20 years, the predominant reason for aortic surgery has switched from aortic dissection to aortic dilatation, while age at surgery has tended to increase (from 32.4 ± 11.9 years to 35.2 ± 12.4 years; P=0.075). Mitral valve surgery was performed in 61 patients (5.6%; six children and 55 adults), including 37 valvuloplasties (60.6%) and 18 mitral valve replacements (29.5%). No significant difference was observed when comparing mitral valve surgery before and after 2000.
  
  
  CONCLUSIONS
  Surgery performed in patients with Marfan syndrome has switched from emergency surgery for aortic dissection to elective surgery for aortic dilatation; this is associated with surgery performed at an older age despite the indication for surgery having decreased from 60mm to 50mm. No significant evolution was observed for mitral valve surgery.
authors.affiliation: Service de cardiologie, centre de référence pour les syndromes de Marfan et apparentés, hôpital Bichat, Paris, France.
authors.forename: Alireza
authors.initials: A
authors.lastname: Samadi
authors.forename: Delphine
authors.initials: D
authors.lastname: Detaint
authors.forename: Carine
authors.initials: C
authors.lastname: Roy
authors.forename: Florence
authors.initials: F
authors.lastname: Arnoult
authors.forename: Gabriel
authors.initials: G
authors.lastname: Delorme
authors.forename: Mathieu
authors.initials: M
authors.lastname: Gautier
authors.forename: Olivier
authors.initials: O
authors.lastname: Milleron
authors.forename: François
authors.initials: F
authors.lastname: Raoux
authors.forename: Catherine
authors.initials: C
authors.lastname: Meuleman
authors.forename: Ulrik
authors.initials: U
authors.lastname: Hvass
authors.forename: Dalil
authors.initials: D
authors.lastname: Hamroun
authors.forename: Christophe
authors.initials: C
authors.lastname: Beroud
authors.forename: Florence
authors.initials: F
authors.lastname: Tubach
authors.forename: Catherine
authors.initials: C
authors.lastname: Boileau
authors.forename: Guillaume
authors.initials: G
authors.lastname: Jondeau
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000784:Aortic Dissection / Q000209:etiology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000209:etiology / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation / Q000639:trends*; D006348:Cardiac Surgical Procedures / Q000639:trends*; D002404:Catheterization / Q000639:trends; D017558:Elective Surgical Procedures; D005260:Female; D006349:Heart Valve Diseases / Q000209:etiology / Q000601:surgery*; D019918:Heart Valve Prosthesis Implantation / Q000639:trends; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D008943:Mitral Valve / Q000601:surgery*; D010297:Paris; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult