Annulo-aortic ectasia with cystic medial necrosis: Diagnosis and surgical treatment☆

Abstract Annulo-aortic ectasia is a term first suggested by Ellis and Cooley for an aneurysmal dilatation of the ascending aorta and aortic annulus frequently associated with aortic regurgitation. The basic structural defect in the aortic wall is cystic medial necrosis which may or may not be accompanied by stigmata of Marfan's syndrome. Thirteen cases fulfilling the above criteria were operated on with four deaths, and three additional cases have been studied. When the patient's diagnosis was uncertain, retrograde angioaortography demonstrated characteristic pear-shaped dilatation of the annulo-aortic region. Surgical treatment consisted of excision of the aneurysm with replacement by a woven Dacron graft and correction of the aortic regurgitation by prosthetic valve.