© Copyright by ICR Publishers 2003 Total Correction of Complete Atrioventricular Septal Defect with Tetralogy of Fallot Edvin Prifti 1,2 , Adrian Crucean 1 , Massimo Bonacchi 2 , Massimo Bernabei 1 , Vincenzo Stefano Luisi 1 , Bruno Murzi 1 , Vittorio Vanini 1 1 Division of Pediatric Cardiac Surgery, G. Pasquinucci Hospital, CREAS-IFC-CNR, Massa, Italy, 2 Cattedra di Cardiochirurgia, University of Careggi, Florence, Italy The association of tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) is a well-recognized congenital heart malformation that occurs in 5-10% of hearts with CAVSD (1-3) and in 1.7% of patients with TOF (2). The complex exhibits features of CAVSD and TOF, specifically a non-restric- tive inlet-type ventricular septal defect (VSD) with anterior extension, an ostium primum, a common unpartitioned atrioventricular (AV) valve, anterosupe- rior deviation of the infundibular septum, and right ventricular outflow tract (RVOT) obstruction (3). Initial experiences of surgical correction of this lesion were associated with high mortality (1,4-7), but more recent reports have demonstrated acceptable outcome (8-16). Controversy persists regarding the surgical approach, which is related to the number of patches employed for CAVSD correction, the use of a right ventriculoto- my, cleft closure and RVOT reconstruction. As yet, the timing of complete repair and the use of a preliminary shunt have not been well defined in the literature. During the past 11 years, the present authors have used a one-patch technique for CAVSD-TOF correc- tion. The aim of the present study was to describe the rationale of the surgical approach used, to explore the best management approach to CAVSD-TOF, and to present results in relation to previously reported series. Clinical material and methods Patients Between January 1990 and September 2001, 17 con- secutive children with CAVSD-TOF underwent complete correction. Children with congenital pul- monary atresia, unbalanced CAVSD and severe hypoplasia of either of the ventricles, and atrioventric- ular or ventriculoarterial discordance were excluded from the study. Three other children who had under- gone palliation and were awaiting complete repair were also excluded. During the same period, 146 Address for correspondence: Massimo Bonacchi MD, Divisione di Cardiochirurgia, Policlinico di Careggi, Viale Morgagni, 85, 50134 Careggi Firenze, Italy Background and aim of the study: The authors’ expe- rience in surgical management of tetralogy of Fallot in association with complete atrioventricular septal defect (CAVSD-TOF) is reported. Methods: Between January 1990 and September 2001, 146 children with CAVSD underwent complete cor- rection. ASD closure was performed using a one-patch technique, with autologous pericardial patch. Seventeen patients presented with CAVSD- TOF, of whom nine (53%) had undergone previous palliation. Mean age at repair was 2.9 ± 1.9 years. The mean gradient across the right ventricular outflow tract (RVOT) was 63 ± 16 mmHg. Six patients (35%) required a transannular patch. Results: Overall in-hospital mortality was 10%. Among patients with CAVSD-TOF, three died in hospital (18%); causes of death were progressive heart failure (n = 2) and multiple organ failure (n = 1). Two patients required mediastinal exploration due to significant bleeding, and two late reoperation for severe left atrioventricular valve regurgitation after intracardiac repair. Mean follow up was 36 ± 34 months. All patients survived and are currently in NYHA class I or II. At follow up, mean gradient across the RVOT was 17 ± 6 mmHg, significantly less than the preoperative value (p <0.001). Conclusion: Complete repair in patients with CAVSD-TOF offers acceptable early and mid-term outcome in terms of mortality, morbidity and reoper- ation rate. Palliation prior to repair may be preferred in cases with small pulmonary arteries, or in severe- ly cyanotic neonates. The RVOT should be managed as for isolated TOF, but a transatrial-transpulmonary approach is the surgery of choice. The Journal of Heart Valve Disease 2003;12:640-648