Total anomalous pulmonary venous connection (TAPVC) is predominantly a neonatal congenital cardiac diagnosis in which the pulmonary veins fail to return to the left atrium. The presence or absence of obstruction defines the clinical presentation, and in the setting of obstructed pulmonary veins, this diagnosis represents a true surgical emergency. Improvements in perioperative care and refinement of surgical techniques continue to allow for improved survival and decreased morbidity. A description of the surgical anatomy and key concepts in the perioperative and surgical management of TAPVC are presented in this review.
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