Does the dilated ascending aorta in an adult with congenital heart disease require intervention?

Objectives: There is increasing attention to prophylactic replacement of the moderately dilated ascending aorta at aortic valve surgery. Moderate ascending aortic dilatation is common in adult patients with conotruncal anomalies. There are no data outlining actual risk of progressive ascending aortic dilatation or dissection to provide management guidelines. Methods: From December 1973 through January 2008, 81 consecutive adults (median age, 34 years; range, 18 - 59 years) with conotruncal anomalies underwent operation on the aortic root, ascending aorta, or aortic valve. Primary cardiac diagnoses included tetralogy of Fallot with or without pulmonary atresia in 60 patients, truncus arteriosus in 12, double-outlet right ventricle in 6, and other in 3. Indications for operation included aortic regurgitation in 69 patients, supracoronary ascending aneurysm in 16, aortic stenosis in 5, and other in 8. Median ascending aortic size was 45 mm (23 - 80 mm). Results: Operations included isolated aortic valve repair/replacement in 63 patients, combined aortic valve replacement and reduction aortoplasty in 9, aortic root replacement in 7, and isolated ascending aortic replacement in 2. Four patients required reoperation during a median follow-up of 3.8 years (maximum 31 years). There were no ascending aortic reoperations after previous reduction aortoplasties or supracoronary ascending aortic grafts, and there were no late aortic dissections. Conclusions: Moderate ascending aortic enlargement is common among patients with conotruncal anomalies coming to operation, but aortic dissection is rare, as is subsequent need for aortic reoperation. Despite current enthusiasm for prophylactic operations on the ascending aorta in patients with acquired disease, these data suggest that the moderately dilated aorta in this setting may be observed.