Pulmonary Atresia With Major Aorto-Pulmonary Collateral Arteries (MAPCAs): Timing and Type of Intervention.

Management of this complex condition has evolved considerably over the past 30 years, transforming the prognosis for this rare and heterogeneous condition. Central to this evolution has been the concept of achieving "unifocalisation" together with the combined approaches of MAPCA recruitment and vascular rehabilitation. Improvements in imaging modalities and applications have transformed the anatomical assessment and understanding of the pulmonary vasculature, which in turn, has facilitated refined surgical techniques and approaches to achieve these improved outcomes. Early assessment enables clinicians to create a precise "road-map" of the pulmonary vasculature, and allow for meticulous planning of the surgical procedures. This article breaks down the management into 6 key decisions that need to be taken in order to achieve the best outcomes across all the variants of the condition.