Criss-cross heart with congenitally corrected transposition: report of a case with d-transposed aorta and ventricular preexcitation.

A case is described of corrected transposition in a situs solitus individual, in which, despite the presence of atrioventricular discordance (1-bulboventricular looping), the aorta was right-sided. A ventricular septal defect and pulmonary stenosis were also present, and the case had been erroneously diagnosed during life as complete transposition. The true diagnosis was not appreciated at surgery, and correction of the anomaly was attempted using a modification of Fontan's operation. The criss-cross atrioventricular connections were only fully appreciated at autopsy. The patient also exhibited ventricular preexcitation of Wolff-Parkinson-White variety type A. Histopathological examination revealed the presence of two atrioventricular nodes in the right atrium, each contacting the ventricular conduction tissue which was distributed to the morphologically appropriate ventricles. In addition, a left-sided accessory atrioventricular connection perforated the anulus of the left-sided, morphologically tricuspid, valve.