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Total anomalous pulmonary venous connection
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.
Additional recommended knowledge
There are four variants:
TAPVC can occur with obstruction, which occurs when the anomalous vein enters a vessel at an acute angle and can cause pulmonary venous hypertension and cyanosis because blood cannot easily enter the new vein as easily.
In TAPVC without obstruction, surgical redirection can be performed within the first month of life. With obstruction, surgery should be undertaken emergently. PGE1 should not be given because a patent ductus arteriosus adds even more volume into the already overloaded pulmonary flow.
|This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Total_anomalous_pulmonary_venous_connection". A list of authors is available in Wikipedia.|