Annuloaortic ectasia in a patient with congenital absence of the left pericardium.

We report a patient with congenital absence of the left pericardium with development of progressive annuloaortic ectasia and aortic insufficiency during a 12-year period. The patient was treated with a Bentall procedure. Pathologic examination of the aorta revealed cystic medial necrosis. The surgical management and a possible association between congenital absence of pericardium and Marfan syndrome are discussed.