Isolated atrioventricular discordance. Report of two surgical cases with isolated ventricular inversion.

Isolated ventricular inversion is a term used for a congenital heart malformation with the segmental arrangement atrioventricular (AV) discordance and ventriculoarterial concordance. It describes a condition which from a physiological point of view resembles complete transposition of the great arteries. We have recently seen two patients with this anomaly. Both underwent intracardiac repair by means of a Mustard operation at 10 years and 10 months of age, respectively. Associated lesions in the first patient were an AV septal defect with two AV orifices (partial AV canal, ostium primum defect), partial anomalous pulmonary venous return, and anomalies in the systemic venous drainage. A perimembranous ventricular septal defect complicated the condition in the second patient.