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.