Aortic valvular malformation associated with coincident cardiovascular anomalies: morphologic considerations.

The association of a bicuspid aortic valve with other congenital cardiac malformations, prominently coarctation of the aorta, is widely accepted with estimates of frequency reported to be 20% overall and ranging between 25-50% to as high as 85% when there is coarctation. The anatomic configuration of the valve has received little comment and, excepting those individuals requiring aortic valve surgery coincident with repair of other malformations, has been limited to postmortem examination. Recently, however, an angiographic technique for accurately evaluating the morphology of the aortic valve has been utilized allowing for the determination of aortic valvular appearance at routine evaluation by cardiac catheterization. We have currently examined 17 individuals with various forms of congenital cardiac malformations in whom an aortic valvular abnormality was suspected as a coexistent abnormality employing the technique of orifice-view aortography. The present report concentrates on the aortic valvular anatomy of these patients.