Intrapulmonary Right Vertical Vein in Supracardiac Total Anomalous Pulmonary Venous Connection with Right Isomerism

A 4-month-old female infant with cyanosis, excessive sweating, and failure to thrive was referred for CT, which showed bilateral trilobed lungs, midline liver, and asplenia consistent with right isomerism. Complex intracardiac defects were seen: dextrocardia, discordant atrioventricular connections, common atrioventricular valve, double outlet right ventricle, and pulmonary stenosis. Left-sided pulmonary veins formed a common vein that crossed over horizontally to the right hemithorax (Fig 1, A and B). This vein had a vertical course within the right lung receiving all right-sided pulmonary veins to drain into the right brachiocephalic vein. Intrapulmonary course of vertical veins in patients with supracardiac total anomalous pulmonary venous connection is an uncommon abnormality. Obstruction of this drainage has been reported in about one-half of patients (1). Cardiac CT accurately depicts the intrapulmonary course of vertical veins (Figs 1, A and B, and 2), which is difficult to visualize at echocardiography, which is usually the initial imaging modality in children with congenital heart defects.

Figure 1:

A, Anterior, B, right anterior oblique, C, right lateral, and, D, posterior view volume-rendered images, with aorta and pulmonary arteries removed, show the common vein (thick arrows) crossing over horizontally to the right side having a vertical intrapulmonary course to drain into the right brachiocephalic vein (thin arrows).

Figure 2:

A, Coronal and, B, axial lung window CT angiography images show the common vein (arrow) crossing over to the right side with a vertical intrapulmonary course to drain into the right brachiocephalic vein. The common vein is seen to be surrounded completely by lung parenchyma.