Anomalous pulmonary venous connection
| Anomalous pulmonary venous connection | |
|---|---|
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| Illustration of total anomalous pulmonary venous connection | |
| Classification and external resources | |
| Specialty | medical genetics |
| ICD-10 | Q26.2-Q26.4 |
| ICD-9-CM | 747.41-747.42 |
| eMedicine | ped/2816 |
Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital defect of the pulmonary veins.
Total anomalous pulmonary venous connection, also known as total anomalous pulmonary venous drainage and total anomalous pulmonary venous return, is a rare cyanotic congenital heart defect in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation. (Normally, pulmonary veins return 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, patent ductui arteriosa 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.
There are four variants: Supracardiac (50%): blood drains to one of the innominate veins (brachiocephalic veins) or the superior vena cava; Cardiac (20%), where blood drains into coronary sinus or directly into right atrium; Infradiaphragmatic (20%), where blood drains into portal or hepatic veins; and a mixed (10%) variant.
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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