Spectrum of aortic operations in 300 patients with confirmed or suspected Marfan syndrome.
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.
LeMaire
Scott A
SA
Cardiovascular Surgery Service, Texas Heart Institute, St. Luke's Episcopal Hospital, Houston, Texas, USA. slemaire@bcm.tmc.edu
Carter
Stacey A
SA
Volguina
Irina V
IV
Laux
Anne T
AT
Milewicz
Dianna M
DM
Borsato
Garry W
GW
Cheung
Catherine K
CK
Bozinovski
John
J
Markesino
Jennifer M
JM
Vaughn
William K
WK
Coselli
Joseph S
JS
D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000601:surgery; D001012:Aorta, Abdominal / Q000601:surgery; D001013:Aorta, Thoracic / Q000601:surgery; D001014:Aortic Aneurysm / Q000601:surgery; D017544:Aortic Aneurysm, Abdominal / Q000601:surgery; D017545:Aortic Aneurysm, Thoracic / Q000601:surgery; D001018:Aortic Diseases / Q000209:etiology / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D002648:Child; D015897:Comorbidity; D005260:Female; D006801:Humans; D007431:Intraoperative Complications / Q000401:mortality; D015993:Life Tables; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D008875:Middle Aged; D011183:Postoperative Complications / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome