Tetralogy of Fallot Repair: The Right Ventricle Infundibulum Sparing (RVIS) Strategy

Despite the excellent operative survival for tetralogy of Fallot (TOF) repair, well-documented long-term complications and reduced life expectancy remain challenges for these patients and their clinicians. In an attempt to change the natural history of repaired TOF, we at Texas Children's Hospital (Houston, TX) have developed a management strategy not focused on age, but rather focused on preserving the right ventricular (RV) infundibulum. The RV infundibulum sparing (RVIS) repair of TOF consists of a transatrial and transpulmonary approach to close the ventricular septal defect and resect RV infundibular muscle coupled with a mini (< 5 mm) transannular patch or no ventricular incision. This strategy is applied with the ambition of decreasing the well-documented, long-term complications of TOF repair with large right ventriculotomies such as RV dilation, arrhythmias, need for pulmonary valve replacement, and RV failure. The RVIS strategy is an attempt based on our current knowledge and experience to optimize the time of repair so that we can not only maximize the early operative results but the long-term effects of this approach as these children mature into adolescents and adults. We have uniformly applied the RVIS strategy since 1995, which includes over 320 isolated TOF patients. We are currently reviewing this cohort in hopes that it will strengthen our beliefs and known results as well as give us more insight into whether the RVIS strategy can change the natural history of repaired TOF.

Keywords: Tetralogy of Fallot, Infundibulum sparing repair, Tetralogy of Fallot repair