Repair of complete atrioventricular septal defect with tetralogy of Fallot

The association of the tetralogy of Fallot (TOF) with a complete atrioventricular septal defect (CAVSD) is a well-recognized congenital heart malformation occurring in 5–10% of hearts with CAVSD and in 1.7% of patients with TOF (1). The complex exhibits features of CAVSD and TOF, specifically a nonrestrictive inlet-type ventricular septal defect (VSD) with anterior extension, an ostium primum, a common unpartitioned atrioventricular valve, anterosuperior deviation of the infundibular septum, and right ventricular outflow tract obstruction (RVOTO) (1). CAVSD associated with TOF (CAVSD-TOF) affects the septa of both atria and ventricles and both ventricular inlets and outlets. Initial experiences of surgical correction for this lesion were associated with high mortality (2-4), however recent reports have demonstrated acceptable outcome (5-14). Controversy still exists about the surgical approach, this related to the number of patches employed for CAVSD correction, the use of a right ventriculotomy, cleft closure and RVOT reconstruction, employment of a transannular patch, previous palliative procedures etc (15,16). The timing of complete repair and the utility of a preliminary shunt have not been well defined in the literature yet.