Long-term results of aortic valve-sparing operations in patients with Marfan syndrome.
OBJECTIVE
The appropriateness of aortic valve-sparing operations in patients with Marfan syndrome has been questioned. This study examines the long-term results of these operations in patients with Marfan syndrome.


METHODS
From 1988 to 2006, 103 consecutive patients with Marfan syndrome (mean age, 37 +/- 12 years) and aortic root aneurysm had aortic valve-sparing operations. Emergency surgery was performed in 11 patients: 8 for acute type A aortic dissection and 3 for unexplained persistent chest pain. Fourteen patients also had mitral valve surgery. The technique of aortic valve reimplantation was used in 77 patients, and aortic root remodeling was used in 26 patients. Patients were followed prospectively and underwent annual echocardiographic studies. The mean follow-up was 7.3 +/- 4.2 years and 100% complete.


RESULTS
There was 1 operative death and 5 late deaths. Four of the 6 deaths were due to complications of aortic dissections. The patients' survival at 15 years was 87.2% compared with 95.6% for the general population of Ontario matched for age and sex. Seven patients had important aortic insufficiency: 4 mild to moderate, 2 moderate, and 1 moderate to severe. Freedom from greater than mild aortic insufficiency at 15 years was 79.2%. Three patients, all after aortic root remodeling, had aortic valve replacement, 2 for aortic insufficiency and 1 for endocarditis. At the most recent follow-up, 97 patients were alive: 86 were in functional class I, and 11 were in functional class II.


CONCLUSIONS
Aortic valve-sparing operations provided excellent clinical outcomes in this series of patients with Marfan syndrome. Postoperatively, complications of aortic dissections were the leading cause of death.
David
Tirone E
TE
Peter Munk Cardiac Centre of Toronto General Hospital, University Health Network and University of Toronto, Toronto, Ontario, Canada. tirone.david@uhn.on.ca
Armstrong
Sue
S
Maganti
Manjula
M
Colman
Jack
J
Bradley
Timothy J
TJ
D000293:Adolescent; D000328:Adult; D000368:Aged; D001014:Aortic Aneurysm / Q000150:complications / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D002648:Child; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality; D008875:Middle Aged; D008943:Mitral Valve / Q000601:surgery; D012086:Reoperation; D015996:Survival Rate; D055815:Young Adult