[Atrioventricular discordance. Clinico-surgical experience 1990-2000]

To know the incidence of ventriculoarterial connections combined with atrioventricular discordance, associated lesions and surgical results, including the first case with anatomical correction.

All patients with atrioventricular discordance by echocardiography from 1990 to March 2000 were analyzed.

Thirty six patients with atrioventricular discordance were found. Ages ranged from 0.1 to 46 years, with a mean 9.2 years (SD 5.9 years). Atrial chambers were situs solitus in 88.9%, inversus in 11.2%. The ventriculoarterial connections were discordant in 28 (77.7%), double outlet right ventricle in 4 (11.1%) (one of them was a "criss cross" heart), single outlet (pulmonary atresia) in 4 (11.1%), and double outlet left ventricle in 1 (2.7%). Associated lesions: Ventricular septal defect with pulmonary stenosis or atresia was present in 21 (58%), ventricular septal defect with no pulmonary obstruction was observed in 10 (28%). Five had tricuspid regurgitation with right ventricular disfunction, (two adults). Surgical results: 22 (61%) required 28 surgical procedures: 8 (36%) were palliative and 19 (86%) were corrective, one of them was our first anatomical correction. Operative mortality in all was 40.1%, postoperative atrioventricular block was observed in 9 (40.1%).

Right ventricular dysfunction is not uncommon. Surgical results revealed high mortality and high pacemaker requirement for atrioventricular block.