Total anomalous pulmonary venous connection overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief:Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [[4]]

Assistant Editor-In-Chief: Kristin Feeney, B.S. [[5]]

Overview

Total anomalous pulmonary venous connection is a rare congenital heart disease, in which an abnormal connection of pulmonary veins with the systemic venous circulation leads to cyanosis in the newborn.

Total anomalous pulmonary venous connection (TAPVC), also known as total anomalous pulmonary venous drainage(TAPVD) and total anamalous pulmonary venous return(TAPVR), is a rare cyanotic congenital heart defect (CHD) in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation. (Normally, pulmonary venous return carries oxygenated blood to the left atrium and to the rest of the body). A patent foramen ovale or an atrial septal defect must be present in order to allow systemic blood flow.