Superoinferior ventricles with criss-cross atrioventricular connections and intact ventricular septum

We report a 6-year-old girl with superoinferior ventricles, criss-cross atrioventricular (AV) relationships with solitus atria, a D-loop, L-transposition of the great arteries, subpulmonary stenosis, but without a ventricular septal defect (VSD). The diagnosis was made by echocardiographic examination and was strengthened by cardiac catheterization and angiocardiography. Balloon atrial septostomy in the neonatal period and a modified Blalock-Taussig shunt at the age of 16 months were performed to alleviate hypoxia. Following the shunt operation, the clinical condition of the patient deteriorated with progressive cardiac enlargement, congestive heart failure, and tricuspid regurgitation. We assume that the absence of a VSD contributed to this deterioration. Subsequent improvement was obtained with balloon enlargement of the atrial septal defect and a bidirectional Glenn anastomosis.