Refers to anatomy: Where pulmonary veins (PVs) connect
Definitions
Total anomalous pulmonary venous return (TAPVR) or "drainage"
Refers to hemodynamics: Where pulmonary venous flow returns (drain)
Failure of connection between PVs & left atrium
Category: Cyanotic; heart size & pulmonary vascularity depend on type
Hemodynamics
All pulmonary venous return goes to right heart (extracardiac left-to-right shunt)
Intracardiac right-to-left shunt through patent foramen ovale (PFO)
All types are admixture lesions
3 types
Supracardiac TAPVR (type I, 50%): "Vertical" common PV joins left innominate vein
Cardiac TAPVR (type II, 20%): Common PV joins coronary sinus or right atrium
Infracardiac TAPVR (type III, 20%): Common PV joins portal vein, ductus venosus, hepatic veins, or inferior vena cava
Mixed or complex TAPVR (type IV) when there is mixture of aforementioned types (10%)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Xiang M et al: Mixed type of total anomalous pulmonary venous connection: diagnosis, surgical approach and outcomes. J Cardiothorac Surg. 15(1):293, 2020
Abdel Razek AAK et al: Computed tomography angiography and magnetic resonance angiography of congenital anomalies of pulmonary veins. J Comput Assist Tomogr. 43(3):399-405, 2019
Fuentes Rojas SC et al: Transcatheter embolization of a persistent vertical vein: a rare cause of left-to-right shunt and right-sided heart failure. Methodist Debakey Cardiovasc J. 15(1):86-7, 2019
Han F et al: The case of the missing pulmonary vein: a focused update on anomalous pulmonary venous connection in congenital cardiovascular disease. Echocardiography. 36(10):1930-5, 2019
White BR et al: Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction. Ann Thorac Surg. 108(1):122-9, 2019
Seller N et al: How many versus how much: comprehensive haemodynamic evaluation of partial anomalous pulmonary venous connection by cardiac MRI. Eur Radiol. 28(11):4598-606, 2018
Tremblay C et al: Sutureless versus conventional pulmonary vein repair: a magnetic resonance pilot study. Ann Thorac Surg. 105(4):1248-54, 2018
Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
Kasai H et al: Adult partial anomalous pulmonary venous connection with drainage to left atrium and inferior vena cava clearly visualized on a combination of multiple imaging techniques. Circ J. 81(10):1547-9, 2017
Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
Bhatia A et al: Infracardiac total anomalous pulmonary venous return: an unusual cause of neonatal portal vein enlargement. World J Pediatr Congenit Heart Surg. 5(1):131-2, 2014
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
Robinson BL et al: Magnetic resonance imaging of complex partial anomalous pulmonary venous return in adults. Circulation. 129(1):e1-2, 2014
Saremi F et al: The art of color coding for three dimensional diagnostic mapping of unusual cardiothoracic vascular abnormalities. Int J Cardiol. 166(3):e68-74, 2013
Seale AN et al: Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg. 145(5):1255-62, 2013
Xu Z et al: [Evaluation of anomalous pulmonary venous connection: comparison between dual-source CT and echocardiography.] Sheng Wu Yi Xue Gong Cheng Xue Za Zhi. 30(2):272-7, 311, 2013
Koplay M et al: Cardiovascular MR imaging findings of total anomalous pulmonary venous connection to the portal vein in a patient with right atrial isomerism. Wien Klin Wochenschr. 124(23-24):848-50, 2012
Lakshminrusimha S et al: Use of CT angiography in the diagnosis of total anomalous venous return. J Perinatol. 29(6):458-61, 2009
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Total anomalous pulmonary venous connection
Refers to anatomy: Where pulmonary veins (PVs) connect
Definitions
Total anomalous pulmonary venous return (TAPVR) or "drainage"
Refers to hemodynamics: Where pulmonary venous flow returns (drain)
Failure of connection between PVs & left atrium
Category: Cyanotic; heart size & pulmonary vascularity depend on type
Hemodynamics
All pulmonary venous return goes to right heart (extracardiac left-to-right shunt)
Intracardiac right-to-left shunt through patent foramen ovale (PFO)
All types are admixture lesions
3 types
Supracardiac TAPVR (type I, 50%): "Vertical" common PV joins left innominate vein
Cardiac TAPVR (type II, 20%): Common PV joins coronary sinus or right atrium
Infracardiac TAPVR (type III, 20%): Common PV joins portal vein, ductus venosus, hepatic veins, or inferior vena cava
Mixed or complex TAPVR (type IV) when there is mixture of aforementioned types (10%)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Xiang M et al: Mixed type of total anomalous pulmonary venous connection: diagnosis, surgical approach and outcomes. J Cardiothorac Surg. 15(1):293, 2020
Abdel Razek AAK et al: Computed tomography angiography and magnetic resonance angiography of congenital anomalies of pulmonary veins. J Comput Assist Tomogr. 43(3):399-405, 2019
Fuentes Rojas SC et al: Transcatheter embolization of a persistent vertical vein: a rare cause of left-to-right shunt and right-sided heart failure. Methodist Debakey Cardiovasc J. 15(1):86-7, 2019
Han F et al: The case of the missing pulmonary vein: a focused update on anomalous pulmonary venous connection in congenital cardiovascular disease. Echocardiography. 36(10):1930-5, 2019
White BR et al: Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction. Ann Thorac Surg. 108(1):122-9, 2019
Seller N et al: How many versus how much: comprehensive haemodynamic evaluation of partial anomalous pulmonary venous connection by cardiac MRI. Eur Radiol. 28(11):4598-606, 2018
Tremblay C et al: Sutureless versus conventional pulmonary vein repair: a magnetic resonance pilot study. Ann Thorac Surg. 105(4):1248-54, 2018
Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
Kasai H et al: Adult partial anomalous pulmonary venous connection with drainage to left atrium and inferior vena cava clearly visualized on a combination of multiple imaging techniques. Circ J. 81(10):1547-9, 2017
Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
Bhatia A et al: Infracardiac total anomalous pulmonary venous return: an unusual cause of neonatal portal vein enlargement. World J Pediatr Congenit Heart Surg. 5(1):131-2, 2014
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
Robinson BL et al: Magnetic resonance imaging of complex partial anomalous pulmonary venous return in adults. Circulation. 129(1):e1-2, 2014
Saremi F et al: The art of color coding for three dimensional diagnostic mapping of unusual cardiothoracic vascular abnormalities. Int J Cardiol. 166(3):e68-74, 2013
Seale AN et al: Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg. 145(5):1255-62, 2013
Xu Z et al: [Evaluation of anomalous pulmonary venous connection: comparison between dual-source CT and echocardiography.] Sheng Wu Yi Xue Gong Cheng Xue Za Zhi. 30(2):272-7, 311, 2013
Koplay M et al: Cardiovascular MR imaging findings of total anomalous pulmonary venous connection to the portal vein in a patient with right atrial isomerism. Wien Klin Wochenschr. 124(23-24):848-50, 2012
Lakshminrusimha S et al: Use of CT angiography in the diagnosis of total anomalous venous return. J Perinatol. 29(6):458-61, 2009
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