Total anomalous pulmonary venous connection classifications

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]

Classifications

There are four variants:

• Supracardiac (50%): blood drains to one of the innominate veins (brachiocephalic veins) or the superior vena cava
• Cardiac (20%): blood drains into coronary sinus or directly into right atrium
• Infradiaphragmatic (20%): blood drains into portal or hepatic veins
• Mixed (10%)

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.