Repair of complete atrioventricular septal defect with tetralogy of fallot: our experience and literature review

OBJECTIVES: The aim of this report is to describe the rationale of our surgical approach, to explore the best management for complete atrioventricular septal defect associated with the tetralogy of Fallot (CAVSD-TOF), and to present our outcome in relation to the previously reported series. MATERIALS AND METHODS: Between January 1990 and January 2002, 17 consecutive children with CAVSD-TOF underwent complete correction. Nine patients (53%) underwent previous palliation. Mean age at repair was 2.9 +/- 1.9 years. Mean gradient across the right ventricular outflow tract was 63 +/- 16 mmHg. All children underwent closure of septal defect with a one-patch technique, employing autologous pericardial patch. Maximal tissue was preserved for LAVV reconstruction by making these incisions along the RV aspect of the ventricular septal crest. LAVV annuloplasty was performed in 10 (59%) patients. Six patients (35%) required a transannular patch. RESULTS: Three (17.6%) hospital deaths occurred in this series. Causes of death included progressive heart failure in two patients and multiple organ failure in the other patient. Two patients required mediastinal exploration due to significant bleeding. Dysrhythmias were identified in 4 of 11 patients undergoing a right ventriculotomy versus none of the patients undergoing a transatrial transpulmonary approach (p = ns). The mean intensive care unit stay was 3.2 +/- 2.4 days. Two patients required late reoperation due to severe LAVV regurgitation at 8.5 and 21 months, respectively, after the intracardiac complete repair. The mean follow-up time was 36 +/- 34 months. All patients survived and are in NYHA functional class I or II. The LAVV regurgitation grade at follow-up was significantly lower than soon after operation, 1.1 +/- 0.4 versus 1.7 +/- 0.5 (p = 0.002). At follow-up, the mean gradient across the right ventricular outflow tract was 17 +/- 6 mmHg, significantly lower than preoperatively (p < 0.001). CONCLUSIONS: Complete repair in patients with CAVSD-TOF seems to offer acceptable early and mid-term outcome in terms of mortality, morbidity, and reoperation rate. Palliation prior to complete repair may be reserved in specific cases presenting small pulmonary arteries or severely cyanotic neonates. The RVOT should be managed in the same fashion as for isolated TOF; however, a transatrial transpulmonary approach is our approach of choice.