Atrioventricular septal defect with tetralogy of Fallot or double-outlet right ventricle: surgical considerations.

The combination of atrioventricular septal defect (AVSD) and tetralogy of Fallot (TOF) constitutes one of the more difficult entities presented to the cardiac surgeon, from both an anatomic and physiological point of view. TOF is encountered in 2.7% to 10% of cases of AVSD, and AVSD complicates 1% to 6.5% of cases of TOF. At least 75% of children with TOF and AVSD have Down's syndrome. The gold standard for rapid noninvasive diagnosis of AVSD with TOF is two-dimensional echocardiography. We prefer to perform elective surgery at 18 to 36 months of age. Our technique is a combination of our patch repair for AVSD and our transatrial transpulmonary repair of TOF. We have performed repair of AVSD with TOF (or double-outlet right ventricle) in 26 patients, of mean age and weight, 34.6 months (2 to 44 months) and 11.8 kg (3 to 37 kg), respectively. There was one hospital death (3.9%, CL = 0% to 20%). Over a follow-up time of 1,650 patient months, there have been two late deaths. Actuarial survival is similar to that for isolated TOF, although freedom from reoperation is significantly worse. Currently, repair of AVSD and TOF is considered a palliative rather than curative operation, but on balance one that has favorably altered the early and midterm outlook for many children.