[Atrioventricular discordance without ventriculo-arterial discordance. Apropos of 3 cases]

Three cases of atrioventricular without ventriculo-arterial discordance are reported. The first case was a young woman with situs solitus, atrioventricular discordance and ventriculo-arterial concordance, large atrial and small ventricular septal defects. The functional tolerance was relatively good. The second case was an infant girl with dextrocardia by dextroversion and abdominal situs solitus. The child had atrioventricular discordance with ventriculo-arterial concordance and an ostium primum atrial septal defect. Cyanosis was mild and, in addition, complete heart block was diagnosed on the fourth day of life. The functional tolerance was good in this case too and growth was normal. The third case was a young girl with levocardia, atrial situs inversus, and abdominal situs inversus. A double outlet right ventricle was diagnosed at catheterisation with atrioventricular discordance, ventricular septal defect, and severe valvular and infundibular pulmonary stenosis. The first palliative procedure was performed at eight months: a left Blalock-Taussig anastomosis. As cyanosis has recurred a second procedure is being considered, the child now being 5 years old. Different forms of atrioventricular discordance without ventriculo-arterial discordance have been described: atrioventricular discordance with ventriculo-arterial concordance; atrioventricular discordance with double outlet right ventricle; atrioventricular discordance with double outlet left ventricle; atrioventricular discordance with a single vessel issuing from the right or left ventricle. With reference to their personal cases and those described in the literature, the authors describe the anatomy, physiopathology and the clinical consequences of the different forms encountered in their cases. The different surgical techniques for each anatomical form of this condition are discussed.