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Total Anomalous Pulmonary Venous Circulation (TAPVC)

Total anomalous venous connections, or TAPVC, is a congenital malformation in which the pulmonary veins join to form a “common vein” but do not attach normally to the left atrium. The Pulmonary venous blood returns to the right atrium by one of three routes. An atrial septal communication must be present to allow blood to reach the left atrium and the left ventricle.

There are three types of TAPVC referred to as supracardiac, intracardiac, or infracardiac type TAPVC. In the supracardiac type, the common “ventricle vein” connects to the superior vena cava system via an anomalous “vertical vein” (see fig. 1). In the intracardiac type, the common pulmonary vein connects to the right atrium directly through the coronary sinus (see fig. 3). In the infracardiac type TAPVC, the common vein connects to the inferior vena cava (IVC) by a vertical vein that passes through the diaphragm (see fig. 4). The blood must pass through the liver to reach the IVC. This form of TAPVC usually causes severe obstructions to flow.

The repair of TAPVC (see fig. 2): The common vein is connected to the left atrium. The anomalous vertical vein is tied off. The ASD is closed (commonly achieved with a patch). Normal blood flow will then be restored.

Figure 1: Supracardiac type TAPVC. Change Common vein to Ventricle vein
Figure 2: Repaired supracardiac type TAPVC.
Figure 3: Intracardiac type TAPVC.
Figure 4: Infracardiac type TAPVC.

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