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An 11-day-old male neonate presented with cyanosis and tachypnea. Cardiac CT angiography revealed the pulmonary veins draining through a single pulmonary venous trunk without communication with the left atrium, descending to the infradiaphragmatic region and connecting to the portal vein, resulting in a portal venous system aneurysm (Figure, Movies 1–3). Surgical repair was promptly performed, connecting the single pulmonary venous trunk to the posterior wall of the left atrium. Currently, he is 9 years old and asymptomatic.
Images in an 11-day-old male neonate with cyanosis and tachypnea. (A–C) Axial and (D) coronal maximum intensity projections and (E) anterior and (F) posterior three-dimensional volume-rendered reconstructions of cardiac CT angiography images show the pulmonary veins draining through a single pulmonary venous trunk (SPVT). The pulmonary veins do not communicate with the hypoplastic left atrium (LA), descending to the infradiaphragmatic region and connecting to the portal vein (PV), resulting in a portal venous system aneurysm. An ostium secundum atrial septal defect (asterisk) and dilated right ventricle (RV) are observed. A = aorta, IVC = inferior vena cava, LIPV = left inferior pulmonary vein, LSPV = left superior pulmonary vein, LV = left ventricle, PV = portal vein, RA = right atrium, RIPV = right inferior pulmonary vein, RMPV = right middle pulmonary vein, RSPV = right superior pulmonary vein.
Movie 1:
Three-dimensional volume-rendered reconstruction of cardiac CT angiography images. IVC = inferior vena cava, LIPV = left inferior pulmonary vein, LSPV = left superior pulmonary vein, RA = right atrium, RIPV = right inferior pulmonary vein, RMPV = right middle pulmonary vein, RSPV = right superior pulmonary vein, SPVT = single pulmonary venous trunk.
Movie 2:
Axial maximum intensity projection of cardiac CT angiography images. IVC = inferior vena cava, LA = left atrium, LIPV = left inferior pulmonary vein, LSPV = left superior pulmonary vein, LV = left ventricle, PV = portal vein, RA = right atrium, RIPV = right inferior pulmonary vein, RMPV = right middle pulmonary vein, RSPV = right superior pulmonary vein, RV = right ventricle, SPVT = single pulmonary venous trunk.
Movie 3:
Coronal maximum intensity projection of cardiac CT angiography images. IVC = inferior vena cava, LIPV = left inferior pulmonary vein, LSPV = left superior pulmonary vein, PV = portal vein, RIPV = right inferior pulmonary vein, RMPV = right middle pulmonary vein, RSPV = right superior pulmonary vein, SPVT = single pulmonary venous trunk.
Total anomalous pulmonary venous connection (TAPVC) represents 1%–5% of all cardiovascular congenital anomalies. It results from persistence of the primitive connections between the pulmonary veins and cardinal systemic veins. Due to failure of the pulmonary veins to connect to the left atrium, anomalous connections form between the pulmonary veins and the right atrium or systemic veins (1,2). TAPVC is associated with high mortality, especially if a right-to-left shunt placement is not surgically performed (1,2). In infracardiac TAPVC, anomalous venous drainage is located below the diaphragm (1–4). Obstruction is almost universally present in the infracardiac type. It can occur extrinsically at the level of the diaphragm or in the hepatic sinusoids (particularly when the venous duct is shut) or intrinsically within the anomalous channel (1–4). Infracardiac TAPVC may also be associated with formation of an aneurysm of the portal venous system and collateral pathways (2).
CT angiography is essential in the preoperative planning and postsurgical follow-up for providing accurate anatomic location, drainage site, areas of stenosis, and the course and caliber of the anomalous vessel. Furthermore, it allows for the performance of three-dimensional reconstructions for advanced spatial and anatomic orientation. Pulmonary vein stenosis is the most important complication in the postoperative period (2).
Footnotes
Authors declared no funding for this work.
Disclosures of conflicts of interest: L.d.P.G.d.F. No relevant relationships. L.d.P.S.B. No relevant relationships. M.C.S. No relevant relationships.
Keywords: Congenital, Pulmonary Veins, Shunts, Cardiac, Pulmonary, Thorax, Vascular, Veins, Anatomy
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