Total Anomalous Pulmonary Venous Return.

HomeRadiologyRecently Published PreviousNext Reviews and CommentaryFree AccessImages in RadiologyTotal Anomalous Pulmonary Venous ReturnHarold Goerne , Prabhakar Shantha RajiahHarold Goerne , Prabhakar Shantha RajiahAuthor AffiliationsFrom the Department of Radiology/Cardiac Imaging, Imaging and Diagnostic Center CID, Guadalajara, Mexico (H.G.); Department of Radiology/Cardiac Imaging, IMSS Western National Medical Center, Belisario Dominguez 1000, Guadalajara, JA 44340, Mexico (H.G.); and Department of Radiology, Mayo Clinic, Rochester, Minn (P.S.R.).Address correspondence to H.G. (email: [email protected]).Harold Goerne Prabhakar Shantha RajiahPublished Online:Feb 28 2023https://doi.org/10.1148/radiol.222085MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractSupplemental material is available for this article.A full-term newborn male presented to the emergency department of the pediatric hospital for tachypnea and cyanosis. Chest radiograph showed pulmonary edema and dextrocardia (Fig, A). CT angiography scan showed all four pulmonary veins joining to form a common vein, which extended inferiorly below the diaphragm before it drained into the inferior vena cava, a type III total anomalous pulmonary venous return (TAPVR) (Fig, B and C; Movie). The patient died during surgical repair.(A) Chest radiograph shows dextrocardia with normal-sized heart with increased pulmonary vascular markings and airspace opacities, indicative of pulmonary edema. (B) Coronal maximum intensity projection and (C) volume rendering from a helical thoracic CT angiography scan show all four pulmonary veins joining to form a common vein (arrows), which extends inferiorly below the diaphragm. There is an aneurysm (curved arrows) due to stenosis in the vein before it drains into the inferior vena cava, a type III total anomalous pulmonary venous return. LIPV = left inferior pulmonary vein, LSPV = left superior pulmonary vein, RIPV = right inferior pulmonary vein, RSPV = right superior pulmonary vein.Download as PowerPointOpen in Image Viewer Get the Flash Player to see this video.Movie: Cinematic rendered reconstructions of thoracic CTA show a type III TAPVR, with all the four pulmonary veins joining to form a common vein which drains into infradiaphragmatic inferior vena cava.Download Original Video (12.6 MB)TAPVR is a rare congenital anomaly in which the entire pulmonary venous system drains into a systemic vein, the right atrium, or the coronary sinus, resulting from persistent embryonic connections between pulmonary and cardinal veins (1,2). Based on the ultimate drainage location, TAPVR is classified as: I, supracardiac; II, cardiac; III, infracardiac; and IV, mixed. Infracardiac is the second-most common type of TAPVR, characterized by drainage below the diaphragm to the portal vein, inferior vena cava, hepatic vein, or azygos system (1,2). Extension of the anomalous vein through the diaphragm makes this type more prone to obstruction, resulting in pulmonary edema with a normal-sized heart (3).Disclosures of conflicts of interest: H.G. No relevant relationships. P.S.R. Royalties or licenses from Elsevier; member of the Radiology: Cardiothoracic Imaging editorial board.References1. Vyas HV, Greenberg SB, Krishnamurthy R. MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants. RadioGraphics 2012;32(1):87–98. Link, Google Scholar2. Hassani C, Saremi F. Comprehensive cross-sectional imaging of the pulmonary veins. RadioGraphics 2017;37(7):1928–1954. Link, Google Scholar3. Ferguson EC, Krishnamurthy R, Oldham SAA. Classic imaging signs of congenital cardiovascular abnormalities. RadioGraphics 2007;27(5):1323–1334. Link, Google ScholarArticle HistoryReceived: Aug 22 2022Revision requested: Oct 10 2022Revision received: Nov 9 2022Accepted: Nov 14 2022Published online: Feb 28 2023 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Recently Published Metrics Altmetric Score PDF download