Between January 1995 and April 1995 Twenty-seven patients (age 15 months to 34 years) with atrioventricular discordance underwent repair of major intracardiac defects. Two of them had ventriculoarterial connection as double outlet left ventricle and the rest of the patients had vent- riculoarterial discordance as congenitally corrertted transposition of the great arteries. Cardiac position was in atrial situs inversus and dextracardia in 4 patients. The most common associated pathologies were VSD in 19 patients (70%), pulmonary stenosis or atresia in 15(55%), ASD in 8(29%) and Tricuspid valve regurgitation in 6(22%).

The VSD was closed through the right atrium in 9 patients, the left ventricle in 10 patients. Pul- monary valvotomy was performed in 6 patients. Valveless extracardiac conduits except one were inserted in 9 patients. Six patients with tricuspid regurgitation received mechanical valve replacement One of them had concomittant Fontan procedure because of the morphologic left ventricular hypoplasia. One patient had additional aortic valve replacement.

Two (7.6%)patients died early postoperatively with low cardiacoutput Five patients of whom three had AV block preoperatively received permanent pacemaker. One of them died at 64 day be cause of pacemaker dysfunction. Eighteen patients were in NYHA class I and 6 in class II during an average follow-up of 52 months (range 4 to 97 months).

Elective repair of major anomalies in association with atrioventricular discordance can be ac complished safely but these patients require careful long-term follow-up in anticipation of late problems like that systemic ventriculary dysfunction, tricuspid regurgitaion, conduit stenosis and late complete atrioventrikular block. We preffer the palliative operations for the patients who may need extracardiac conduits under the four years of age.