Total Anomalous Pulmonary Venous Connection (TAPVC)

Sometimes called total anomalous pulmonary venous return (TAPVR).

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With total anomalous pulmonary venous connection the pulmonary veins (veins carrying red, oxygenated blood back from the lungs to the heart) return from the lungs but don’t attach to left atrium. Instead, they go back to the right atrium through abnormal vein connections. This leads to a large left to right shunt, mixing of blood and cyanosis in a newborn infant.  Some newborns can be extremely ill if this connection is narrowed or obstructed.  They can have very low oxygen saturations and can be in severe shock with acidosis. Newborns with obstructed TAPVC require an emergent operation.

Surgical correction:  Consists of sewing the pulmonary veins to the back of the left atrium.  The atrial septal defect (opening between the upper two chambers) is also closed, usually with a patch.  The abnormal vein connecting the pulmonary veins to the regular veins is tied off once the new connection is made.