Discordant Atrioventricular Connections 3: Congenitally Corrected Transposition with Pulmonary Atresia

In one-third of patients with congenitally corrected transposition, there can be co-existing pulmonary stenosis. As explained, although it is stated that no heart exists in the Idriss archive with this lesion, this is not the case, and such a heart in shown in the next video clip. In this video, however, we show the extreme form of obstructed outflow to the pulmonary circulation, namely pulmonary atresia. We demonstrate two examples. The first has a co-existing ventricular septal defect, with the pulmonary arteries fed through a persistently patent arterial duct. In the second heart, in contrast, the ventricular septum is intact. In the latter specimen, because of the discordant connections, it is the morphologically left ventricle that is grossly hypoplastic, and there is an imperforate mitral valve blocking the right-sided atrioventricular connection. We then show a third heart in which there is aortic atresia. In the usual situation, with concordant atrioventricular connections, aortic atresia is usually part of the hypoplastic left heart syndrome. As we saw in the second heart shown in this clip, however, in congenitally corrected transposition, when the ventricular septum is intact, hypoplasia of the left ventricle is associated with pulmonary atresia. The third heart shows that aortic atresia, in the setting of congenitally corrected transposition, can be associated with dilation of the right ventricle, due in the heart shown to dyplasia of the leaflets of the tricuspid valve.