Aortic Valve-Sparing Operations in Marfan Syndrome
What are the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome?
At a large referral center, all patients with Marfan syndrome operated on for aortic root aneurysm from 1998 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank comparisons.
A total of 146 patients with Marfan syndrome had aortic valve-sparing operations during the study interval. Re-implantation of the aortic valve was performed in 121, and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years, and two thirds were men. Before surgery, nine patients had acute type A aortic dissection, two had chronic type A dissection, and three had chronic type B aortic dissection. There was one operative death and six late deaths; five deaths were due to complications of dissection. Mortality rate at 15 years was 6.8 ± 2.9%, higher than that expected in a general population matched for age and sex. Reoperation was required in five patients: two for infective endocarditis, and three for aortic insufficiency (AI). After surgery, three patients developed severe AI, four developed moderate AI, and three mild-to-moderate AI; the rate of AI at 15 years was 7.9 ± 3.3%, and was lower after aortic valve re-implantation than after aortic remodeling. New distal aortic dissections occurred during follow-up in nine patients; the rate of aortic dissection at 15 years was 16.5 ± 3.4%.
The authors concluded that aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications during long-term follow-up. Residual and new aortic dissections were the leading cause of death.
In centers with advanced expertise on the management of patients with aortic disease, aortic valve-sparing operations have become the preferred surgical approach to treat young patients with aortic root aneurysm. Although the presence of abnormal aortic valve fibrillin among patients with Marfan syndrome could lead to less durable results of aortic valve-sparing procedures, these data—from the surgeon who has defined many of the techniques for valve-sparing aorta repair—suggest that, in the correct hands, valve-sparing aorta operations can be used successfully and durably among patients with Marfan syndrome.
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