Long-Term Outcome of Patients With Complete Atrioventricular Septal Defect Combined With the Tetralogy of Fallot: Staged Repair Is Not Inferior to Primary Repair

Abstract

Cyanotic and very young children with complete atrioventricular septal defect combined with the tetralogy of Fallot who require urgent treatment have long-term outcomes after staged repair similar to those of patients who undergo primary repair. Preoperative atrioventricular valve regurgitation is associated with increased risk for reoperation over the long-term.

Contemporary Outcomes After Repair of Isolated and Complex Complete Atrioventricular Septal Defect

“…Despite improvements in contemporary mortality rates after CAVSD repair, reoperation remains a significant challenge. The reintervention rates in this series are similar to the 10% to 20% rate at 10 years reported by other centers for isolated CAVSD [4,5,13] and 20% to 39% rate for series with complex anatomy [6,7,16,17]. Shuhaiber and colleagues [7] found that RV-PA conduit replacements were the most common reoperation in patients with complex CAVSD.…”

“…and 1 late mortality in a series of 16 patients with CAVSD-DORV at a median follow-up of 5.5 years; they concluded that acceptable early results could be accomplished. Vitanova and colleagues [16] reported 47 patients with CAVSD-TOF undergoing repair over a 40-year period, and found a perioperative mortality of 4% and that 10-year survival was around 80%. Our results compare favorably to these and other studies [6,7].…”

Repair of complete atrioventricular septal defect with tetralogy of Fallot

“…Performing primary repair of CAVSD-TOF when the patient is older has potential advantages, (14). The repair of TOF often results in pulmonary valve insufficiency or residual stenosis.…”

“…Initially, in our experience, the children with CAVSD-TOF underwent palliation as a first choice procedure, and later on a complete repair. The arguments for palliation and delayed repair include an easier atrioventricular valve's reconstruction and placement of a larger valved conduit if needed (14). However, palliation carries potential complications, including prolonged cyanosis, excessive ventricular hypertrophy, volume loading of the ventricle, and progression of the atrioventricular valves' regurgitation.…”

“…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)(3)(4), however recent reports have demonstrated acceptable outcome (5)(6)(7)(8)(9)(10)(11)(12)(13)(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).…”