Corrected transposition with severe intracardiac deformities with Wolff-Parkinson-White syndrome in a child. Electrophysiologic investigation and surgical correction

Abstract

This is a report of a 10-year-old child who underwent surgery for complex congenital disease consisting of corrected transposition of the great vessels, ventricular septal defect, patent ductus arteriosus, severe left-sided atrioventricular (AV) valve insufficiency (Ebstein's deformity) and Wolff-Parkinson-White syndrome. The site of his accessory AV connection was localized preoperatively at a left anteriolateral site by isopotential body surface maps and by intracardiac electrophysiological studies. He successfully underwent surgery for closure of the ventricular septal defect, ligation of the patent ductus arteriosus, replacement of the left-sided AV valve, and interruption of the accessory AV pathway. Unavoidable complete AV block acquired at surgery required subsequent permanent pacemaker therapy.