Anatomy of common atrioventricular junction with complex associated lesions.

The essence of the lesion increasingly described as atrioventricular septal defect is the presence of a common atrioventricular junction. In most instances, the common junction is itself shared in more or less equal fashion between the cardiac chambers, producing the so-called balanced arrangement, which can be considered the default option. Complexity can be produced at various levels within this standard lesion. The most complex malformations are seen in the setting of visceral heterotaxy. Greatest complexity is seen with right isomerism. This always includes totally anomalous pulmonary venous connection, even when the pulmonary veins return to the heart. Still further complexity is often added by the presence of pulmonary stenosis or atresia. Imbalance can involve either the atrial or ventricular chambers. Imbalance at atrial level produces one form of double outlet atrium, but typically with balanced ventricles. Ventricular imbalance represents spectrums extending either to double inlet left or right ventricle through a common atrioventricular valve. Complexity at the level of the ventriculoarterial junctions is seen in the form of abnormal ventriculoarterial connections, notably tetralogy of Fallot or double outlet right ventricle. In these settings, the superior bridging leaflet is free-floating. Hypoplasia of the left atrioventricular valve is part of right ventricular dominance and is often associated with the so-called parachute malformation. Dual orifice is also a problem. In both these lesions, the zone of apposition between the bridging leaflets is the effective inlet to the left ventricle.