Background .– Atrioventricular discordance with ventriculo-arterial concordance is a rare cardiac defect whose pathophysiology resembles transposition of the great arteries.

Objective .– To report a series of ten patients with atrioventricular discordance with ventriculo-arterial concordance focusing on segmental analysis, diagnostic difficulties, surgical management and follow-up.

Methods .– Retrospective review of medical files of all patients with this diagnosis seen from 1983 to 2013 in a single institution.

Results .– Seven patients had {I, D,S} segmental arrangement, two had anatomically corrected malposition {S,L,D} and one had {S,L,S} arrangement. Only five patients were correctly diagnosed preoperatively while diagnosis had to be completed during or after surgery in the other five. Mean age at repair surgery or at last surgical intervention was 3.4 years (range: 5 months–12.8 years). Repair surgery finally performed was atrial switch procedure of Senning or Mustard type in eight of 10 patients. Repair included ventricular septal defect closure in three cases and right ventricle to pulmonary artery conduit or patch in four cases. Main postoperative complications were two cases of baffle obstruction requiring reintervention and one sick sinus syndrome needing pacemaker implantation. There was no postoperative heart block. There were two early postoperative deaths and eight late survivors. Mean follow-up after repair was 6.8 years (range: 5 months–25.4 years) with good functional status in all but one patient who will be listed for heart transplantation.

Discussion .– Preoperative diagnosis of atrioventricular discordance with ventriculo-arterial concordance remains challenging. Atrial switch procedure is the surgical method of choice. If correctly diagnosed, long-term follow-up is encouraging. Rhythm disturbances and baffle obstruction are the main postoperative problems in this series.