Total (TAPVC) and partial (PAPVC) anomalous pulmonary venous connection
Semantic difference for more complex heart disease differentiating between how veins are connected and where they ultimately return
Synonyms
Total anomalous pulmonary venous drainage (TAPVD)
Total veins
Definitions
TAPVR means that all pulmonary veins (PVs) connect to location other than left atrium (LA), typically to systemic veins and ultimately right atrium (RA)
Supracardiac TAPVR
Drainage is cephalad, typically via vertical vein to innominate vein
Cardiac TAPVR
Drainage is directly to heart, typically via coronary sinus
Infracardiac TAPVR
Drainage is caudad, coursing below diaphragm
Draining vein passes through liver (portal venous system) before returning to heart
Confluence frequently vertical, long, and thin; can be particularly difficult to identify in fetus
Mixed TAPVR
Drainage of each vein may be to different location or groups of veins may drain to different locations
e.g., 2 right veins may drain to supracardiac location and 2 left veins may drain below diaphragm
Partial anomalous pulmonary venous return (PAPVR)
1 or 2 PVs with anomalous drainage; others drain normally to LA
IMAGING
General Features
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Sinha M et al: Type A3 truncus arteriosus with infracardiac total anomalous pulmonary venous return and single ventricle physiology: a triad of tribulations. J Cardiovasc Comput Tomogr. 14(6):e137-8, 2020
Bakker MK et al: Prenatal diagnosis and prevalence of critical congenital heart defects: an international retrospective cohort study. BMJ Open. 9(7):e028139, 2019
Domadia S et al: Neonatal outcomes in total anomalous pulmonary venous return: the role of prenatal diagnosis and pulmonary venous obstruction. Pediatr Cardiol. 39(7):1346-54, 2018
Paladini D et al: Prenatal diagnosis of total and partial anomalous pulmonary venous connection: multicenter cohort study and meta-analysis. Ultrasound Obstet Gynecol. 52(1):24-34, 2018
Koneti NR et al: Surgical correction of obstructed total anomalous pulmonary venous return soon after birth. Ann Pediatr Cardiol. 8(3):255-6, 2015
Ganesan S et al: Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views. J Ultrasound Med. 33(7):1193-207, 2014
Kawazu Y et al: 'Post-LA space index' as a potential novel marker for the prenatal diagnosis of isolated total anomalous pulmonary venous connection. Ultrasound Obstet Gynecol. 44(6):682-7, 2014
Pinto NM et al: Barriers to prenatal detection of congenital heart disease: a population-based study. Ultrasound Obstet Gynecol. 40(4):418-25, 2012
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Total anomalous pulmonary venous return (TAPVR)
Total (TAPVC) and partial (PAPVC) anomalous pulmonary venous connection
Semantic difference for more complex heart disease differentiating between how veins are connected and where they ultimately return
Synonyms
Total anomalous pulmonary venous drainage (TAPVD)
Total veins
Definitions
TAPVR means that all pulmonary veins (PVs) connect to location other than left atrium (LA), typically to systemic veins and ultimately right atrium (RA)
Supracardiac TAPVR
Drainage is cephalad, typically via vertical vein to innominate vein
Cardiac TAPVR
Drainage is directly to heart, typically via coronary sinus
Infracardiac TAPVR
Drainage is caudad, coursing below diaphragm
Draining vein passes through liver (portal venous system) before returning to heart
Confluence frequently vertical, long, and thin; can be particularly difficult to identify in fetus
Mixed TAPVR
Drainage of each vein may be to different location or groups of veins may drain to different locations
e.g., 2 right veins may drain to supracardiac location and 2 left veins may drain below diaphragm
Partial anomalous pulmonary venous return (PAPVR)
1 or 2 PVs with anomalous drainage; others drain normally to LA
IMAGING
General Features
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Sinha M et al: Type A3 truncus arteriosus with infracardiac total anomalous pulmonary venous return and single ventricle physiology: a triad of tribulations. J Cardiovasc Comput Tomogr. 14(6):e137-8, 2020
Bakker MK et al: Prenatal diagnosis and prevalence of critical congenital heart defects: an international retrospective cohort study. BMJ Open. 9(7):e028139, 2019
Domadia S et al: Neonatal outcomes in total anomalous pulmonary venous return: the role of prenatal diagnosis and pulmonary venous obstruction. Pediatr Cardiol. 39(7):1346-54, 2018
Paladini D et al: Prenatal diagnosis of total and partial anomalous pulmonary venous connection: multicenter cohort study and meta-analysis. Ultrasound Obstet Gynecol. 52(1):24-34, 2018
Koneti NR et al: Surgical correction of obstructed total anomalous pulmonary venous return soon after birth. Ann Pediatr Cardiol. 8(3):255-6, 2015
Ganesan S et al: Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views. J Ultrasound Med. 33(7):1193-207, 2014
Kawazu Y et al: 'Post-LA space index' as a potential novel marker for the prenatal diagnosis of isolated total anomalous pulmonary venous connection. Ultrasound Obstet Gynecol. 44(6):682-7, 2014
Pinto NM et al: Barriers to prenatal detection of congenital heart disease: a population-based study. Ultrasound Obstet Gynecol. 40(4):418-25, 2012
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