Echocardiographic recognition of atrioventricular valve stenosis associated with endocardial cushion defect: Pathologic and surgical correlates

The association of either mitral or tricuspid stenosis with endocardial cushion defects has been well recognized pathologically but is infrequently diagnosed clinically. M mode echocardiographic features such as markedly disproportionate ventricular size, abnormal mitral or tricuspid diastolic echoes or failure to define adequately an atrioventricular (A-V) valve are strongly suggestive of associated stenosis of that valve. In this study mitral stenosis in association with endocardial cushion defect was correctly diagnosed in four of five patients. In the fifth patient (the first seen) the condition was easily diagnosed retrospectively. Tricuspid stenosis was similarly diagnosed prospectively in three of four patients. Significant stenosis was not overlooked or misdiagnosed in any patient with an endocardial cushion defect.

Significant stenosis of an A-V valve in association with endocardial cushion defect carries a high mortality. It is important to recognize the combined lesion clinically because infants with it are not suitable for total surgical correction. Palliative surgical procedures with aggressive medical therapy probably offer the infant the best chance of surviving until ventricular growth may render corrective surgery feasible.