Recognition of imperforate atrioventricular valves by two dimensional echocardiography

The majority of hearts in which selective right or left atrial angiography shows no direct communication between one atrium and a ventricular chamber are considered to be examples of atresia of the right or left atrioventricular valves. Most patients presenting with the clinical features of tricuspid atresia have an absent right atrioventricular connection, while those with features of mitral atresia and a normal aortic root frequently have an absent left atrioventricular connection. By studying 67 patients using two dimensional echocardiography we have identified 12 in whom there was an imperforate atrioventricular valve and not an absent atrioventricular connection. Thus, in 44 hearts with the angiographic features of tricuspid atresia, 36 had absence of the right atrioventricular connection and six had an imperforate right atrioventricular valve. In the latter, four were examples of atrioventricular concordance and two were univentricular hearts of left ventricular morphology in which the mode of atrioventricular connection was through one perforate and one imperforate valve. Similarly, 17 out of 23 cases of mitral atresia had absence of the left atrioventricular connection. The remaining six had an imperforate left atrioventricular valve which was associated with atrioventricular concordance in two patients and with double inlet univentricular heats in four patients.