Infracardiac type total anomalous pulmonary venous return with obstruction and dilatation of portal vein

Abstract

Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection, is a congenital cardiovascular malformation that presents itself in the neonatal period, with cyanosis and tachypnea. There are 4 types of TAPVR with the mixed type being the least common. Any type of TAPVR may be associated with obstruction as result of flow redirection through the liver parenchyma before it may return to the heart, but infracardiac is the most common one. We report a case of a 10-hour-old female, with a mixed (cardiac and infracardiac) TAVPR with obstruction, that showed drainage to the coronary sinus and the portal vein, as the other classic findings in TAVPR, made with computed tomography angiography and echocardiogram. The patient was taken to surgical repair, but unfortunately died during the procedure because of multiple complications.

Keywords: Computed tomography; Congenital; Pulmonary venous anomalies; Total anomalous pulmonary venous return.

Fig. 1

Coronal maximum intensity-projection computed tomography (CT) image shows pulmonary veins drain through a common trunk into the left branch of the porta, which is dilated, configuring findings of infracardiac TAVPR.

Fig. 2

Tridimensional reconstruction (3D) CT image in posteroanterior view shows pulmonary veins drain through a common trunk.

Fig. 3

Tridimensional reconstruction (3D) CT image in posteroanterior view shows pulmonary veins drain through a common trunk below the diaphragm.

Fig. 4

Axial 4 chambers projection CT image shows ostium secundum atrial septal defect and hypoplastic left atrium.

Fig. 5

Tridimensional reconstruction (3D) CT image in posteroanterior view shows a patent ductus arteriosus, hypoplastic ascending aorta and aortic arch and also a common origin of the brachiocephalic trunk and the left common carotid.