Thoracic aortic aneurysm associated with congenital bicuspid aortic valve

Congenital bicuspid aortic valve is a relatively rare malformation. It is reported that the presence of this anomaly predisposes the patient to development of true aortic aneurysms or dissecting aortic aneurysms. Between 1981 and August 1997, 25 patients with an aneurysm of the thoracic aorta associated with congenital bicuspid aortic valve underwent surgical treatment at the authors' institution. There were 20 males and five females. The age of the patients ranged from 27 to 74 years (mean 53 years). There were 18 patients with true ascending aortic aneurysms (of which 10 presented with annulo-aortic ectasia) and seven with dissecting aortic aneurysms (four with DeBakey type I dissection, two with type II and one with type IIIb). These 25 patients constituted 2.6% (25/973) of all cases of surgical operations for aneurysms in the thoracic aorta. Aortic valve dysfunction was noted in 20 patients. The authors performed a valved conduit operation in nine patients, aortic valve replacement and wrapping of the ascending aorta in six, graft replacement of the ascending aorta in five, graft replacement of the ascending aorta and aortic arch in four, and graft replacement of the descending aorta in one. No hospital deaths occurred in the authors' patients. Pathological examination of surgical specimens of the aortic wall showed cystic medial necrosis in 11 patients and mucoid degeneration in nine. In patients with congenital bicuspid aortic valve, attention should be paid to aneurysmal dilatation and aortic dissection as complications in addition to valve dysfunction.