{"id":275,"date":"2018-02-15T19:38:20","date_gmt":"2018-02-15T19:38:20","guid":{"rendered":"https:\/\/wp.uthscsa.edu\/ct-surgery\/?page_id=275"},"modified":"2022-05-31T15:19:51","modified_gmt":"2022-05-31T20:19:51","slug":"total-anomalous-pulmonary-venous-return-tapvr","status":"publish","type":"page","link":"https:\/\/lsom.uthscsa.edu\/ct-surgery\/patient-care\/congenital-heart\/conditions-we-treat\/total-anomalous-pulmonary-venous-return-tapvr\/","title":{"rendered":"Total Anomalous Pulmonary Venous Return (TAPVR)"},"content":{"rendered":"<div class=\"wpb-content-wrapper\"><p>[vc_row][vc_column][vc_column_text]In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus.\u00a0 An ASD is necessary to deliver oxygenated blood to the left heart.\u00a0 TAPVR is classified as supracardiac, cardiac, or infracardiac.\u00a0 In the most common variant of\u00a0<em>supracardiac<\/em>\u00a0TAPVR, all four pulmonary veins drain into a common, left vertical vein, which then drains into the innominate vein, azygous vein, SVC, or RA. . The classic CXR finding is that of a \u201csnowman\u201d or \u201cfigure-of-eight\u201d due to the large vertical vein on the left and SVC on the right\u00a0 In the\u00a0<em>cardiac<\/em>\u00a0subtype, pulmonary venous drainage is into the coronary sinus.\u00a0 In the\u00a0<em>infracardiac<\/em>\u00a0variant, a vertical vein descends below the diaphragm, where it usually connects with the portal vein and then into the IVC.<\/p>\n<p>In cases without obstruction there is a large left-to-right shunt and patients present with heart failure months after birth up to early childhood. \u00a0There may be a gallop or faint systolic murmur secondary to increased flow across the tricuspid valve, and a prominent fixed S2. Patients with non obstructed TAPVR are repaired electively when diagnosed, usually when symptoms develop around 6 months of age. When there is pulmonary venous obstruction, infants present in extremis within hours to days of birth; they are profoundly cyanotic and in severe congestive heart failure.\u00a0 There is marked pulmonary vasculature and edema on CXR.\u00a0 Diagnosis and mapping of the anomalous connections is possible by echocardiography.\u00a0 Obstructed TAPVR is a surgical emergency.\u00a0 Patients require intubation with 100% oxygen, hyperventilation, and correction of acidosis.\u00a0 ECMO has been used as a temporizing measure. 5-15% of patients develop recurrent pulmonary venous obstruction within the first year post-op.<img loading=\"lazy\" decoding=\"async\" class=\"size-full wp-image-276 aligncenter\" src=\"https:\/\/lsom.uthscsa.edu\/ct-surgery\/wp-content\/uploads\/sites\/32\/2018\/02\/families-tapvr.jpg\" alt=\"\" width=\"489\" height=\"302\" srcset=\"https:\/\/lsom.uthscsa.edu\/ct-surgery\/wp-content\/uploads\/sites\/32\/2018\/02\/families-tapvr.jpg 489w, https:\/\/lsom.uthscsa.edu\/ct-surgery\/wp-content\/uploads\/sites\/32\/2018\/02\/families-tapvr-300x185.jpg 300w\" sizes=\"auto, (max-width: 489px) 100vw, 489px\" \/>[\/vc_column_text][\/vc_column][\/vc_row]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>[vc_row][vc_column][vc_column_text]In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus.\u00a0 An ASD is necessary to deliver oxygenated blood to the left heart.\u00a0 TAPVR is classified as supracardiac, cardiac, or infracardiac.\u00a0 In the most common [&hellip;]<\/p>\n","protected":false},"author":161,"featured_media":0,"parent":1337,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-275","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.3 - 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