Total anomalous pulmonary venous connection

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Total anomalous pulmonary venous connection
Classification and external resources
ICD-10 Q26.2
ICD-9 747.41

Total anomalous pulmonary venous connection (TAPVC), also known as total anomalous pulmonary venous drainage (TAPVD) and total anomalous 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 from the lungs to the left atrium where it can then be pumped to the rest of the body). A patent foramen ovale or an atrial septal defect must be present, or else the condition is fatal due to a lack of systemic blood flow.

In some cases, it can be detected prenatally.[1]


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 enter the new vein as easily.

Clinical manifestation


In TAPVC without obstruction, surgical redirection can be performed within the first month of life. With obstruction, surgery should be undertaken emergently. PGE1 should be given because a patent ductus arteriosus allows oxygenated blood to go from the circulation of the right heart to the systemic circulation.


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External links

pl:Całkowite nieprawidłowe przyłączenie żył płucnych
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