How and When Should Tetralogy of Fallot be Palliated Prior to Complete Repair?

Abstract

The controversy regarding the best or ideal surgical management of Tetralogy of Fallot (ToF) stems from the recognition of there being a spectrum of morphology and associated lesions, each of which require a different approach to achieve the three goals of minimizing mortality preserving right ventricular function long-term and minimizing reinterventions. A one-size-fits-all approach to ToF needs to be replaced by a considered and personalized approach in order to yield the best outcomes possible for individual patients. The great majority of patients with ToF undergo primary complete repair between age 3–9 months with excellent outcomes. However, the greatest challenge is the severely cyanotic neonates where primary repair is still associated with high mortality and reintervention rates. Risk factors are low weight and small/poorly developed pulmonary vasculature. High-risk neonates have better outcomes with palliation—but mortality is still high. Palliative interventions in the catherization lab are showing better outcomes than traditional BT shunt and the RVOT stent is emerging as potential game-changer. Primary neonatal repair is still recommended if weight >3 kg and Nakata >100 mm²/m². However, neonates with low weight, small pulmonary arteries or multiple comorbidities (including ToF/AVSD and anomalous LAD) may do better with a staged approach, There is good argument for RVOT stenting as a bridge to complete repair due to its stable circulation without diastolic run off and volume loading of the circulation, and its potential to allow branch PA growth.