Abstract

This review provides an up to date overview of the treatment options for newborns with pulmonary atresia/VSD/MAPCAs. It answers the question of how best to diagnose and formulate a surgical plan for this diverse and heterogeneous patient population. The surgical repair of patients with pulmonary atresia/VSD/MAPCAs can be performed safely with excellent results as a single stage procedure and in the neonatal period in specific circumstances. Pulmonary atresia/VSD/MAPCAs is a complex and varied lesion. Its clinical presentation and management vary between patients and is mainly dictated by the anatomic characteristics of the major aortopulmonary collaterals which constitute the extracardiac sources of pulmonary blood flow. A rigorous evaluation of the patient’s anatomy is mandatory. Precise surgical planning is a prerequisite and exact surgical execution is required for a successful patient outcome.

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