The importance of localizing pulmonary veins in atrial septal defect closure!

Abstract

An 8-year-old girl was admitted for a simple closure of echocardiographically diagnosed Atrial Septal Defect (ASD). During the operation the right pulmonary veins orifices were not detected in the left atrium and attempt to localize them led to the discovery of three additional anomalies, namely Interrupted Inferior Vena Cava (IIVC), Scimitar syndrome, and systemic arterial supply of the lung. Postoperatively these finding were confirmed by CT angiography. This case report emphasizes the need for adequate preoperative diagnosis and presents a very rare constellation of four congenital anomalies that to the best of our knowledge is not reported before.

Figure 1

Intraoperative view of the two anomalous blood vessels. A - The long arrow shows the 12 mm vessel originating below the hilum of the right lung (hollow arrow) after being transferred to the right atrium. The small arrow shows the 7 mm vessel passing through the right dome of the diaphragm (*). B - Inside the right atrium is shown. The large arrow depicts the orifice of the redirected 12 mm vessel, which is fixed to the right atrium just at the right side of the ASD (small arrow). A pericardial patch is used later to redirect flow from this new orifice towards the left atrium

Figure 2

A - Frontal projection of the venous phase of 3D CT angiography with volume rendering, which is obtained after the operation. Annotated structures are: short solid arrow = enlarged azygos vein; long solid arrow = IVC, which is interrupted at the hepatic level; short hollow arrow = SVC; long hollow arrow = Redirected anomalous pulmonary vein; arrow head = hepatic vein, which drains into right atrium. B - The arterial phase depicts the anomalous systemic artery (arrow) arising from the celiac trunk and intending to supply the base of the right lung, which is ligated at the level of the diaphragm.