Current Surgical Management of Ascending Aortic Aneurysm in Children and Young Adults

Background: The low incidence of aortic aneurysm among children and young adults limits information about etiology, surgical indications, procedures of choice, and operative results. Methods: From 2003 to 2008, 35 patients aged 7 to 35 years (mean 21) underwent replacement of the ascending aorta for 19 ascending aortic aneurysms and 16 aortic root aneurysms. Underlying diseases included 10 congenital aortic stenoses, 10 bicuspid aortic valves, 8 congenital heart diseases, 6 Marfan syndromes, and 1 aortitis. Results: Indications for surgery were maximal diameter of 200% of normal for isolated aneurysms, and 160% of normal in case of associated aortic valve dysfunction or symptoms. Operative procedures included 27 ascending aortic replacements with or without aortic valve replacement (including 22 conduits) and 8 valve-sparing operations, performed in 6 patients with Marfan syndrome and 2 with congenital heart diseases. There was 1 in-hospital death. Thirty-four patients survived the operation and are in New York Heart Association functional class II or less at a maximum of 5 years of follow-up. All patients are free from reoperation, but 1 patient had a thromboembolic event. Aortic valve function is good in all 8 patients after the valve-sparing operation. Conclusions: Ascending aortic aneurysm in children and young adults was surgically treated with excellent midterm outcome. A diameter of 200% of normal was the indication for surgery; however, in case of associated lesions, smaller diameters should be considered for surgery. Conduit implantation is the gold standard. Valve-sparing operation can be performed in selected patients with encouraging results.