| 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]
Contents |
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.
In TAPVC without obstruction, surgical redirection can be performed within the first month of life. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed. 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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