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.

Abbreviation and Acronym: AI, aortic insufficiency

CTSNet classification: 18