Twisted atrioventricular connections usually occur in hearts with biventricular artioventricular connections. Here, we describe a case of twisted atrioventricular valves associated with double inlet left ventricle and discordant ventriculo-arterial connections. Color Doppler echocardiography, and cine magnetic resonance imaging, clearly demonstrated that the right atrioventricular valve was located anterior and superior to the left atrioventricular valve, and that the axes of the two atrioventricular valves crossed each other within the dominant left ventricle.

Twisted atrioventricular connections occur almost exclusively in the hearts with biventricular atrioventricular connections. Only one example of double inlet left ventricle has been illustrated in which the axes of the two atrioventricular valves crossed each other. We describe herein three patients, and one autopsied specimen, with double inlet right ventricle in which magnetic resonance imaging clearly demonstrated twisted atrioventricular connections.

We present an example of corrected transposition with twisted discordant atrioventricular connections in which both fetal and postanatal echocardiograms were obtained. We correlate the fetal echocardiograms with the postanatal echocardiograms, placing emphasis on the echocardiographic clues to the diagnosis which were present in fetal life. The diagnosis should be suspected when the cardiac chambers and greatarteries show an unexpected spatial relationship for the given segmental connections, and when the axes of opening of the atrioventricular valves are not parallel.