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Total Anomalous Pulmonary Venous Return
Randy R. Richardson, MD
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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

                                    1. Xiang M et al: Mixed type of total anomalous pulmonary venous connection: diagnosis, surgical approach and outcomes. J Cardiothorac Surg. 15(1):293, 2020
                                    2. 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
                                    3. 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
                                    4. 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
                                    5. White BR et al: Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction. Ann Thorac Surg. 108(1):122-9, 2019
                                    6. 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
                                    7. Tremblay C et al: Sutureless versus conventional pulmonary vein repair: a magnetic resonance pilot study. Ann Thorac Surg. 105(4):1248-54, 2018
                                    8. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                    9. 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
                                    10. Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
                                    11. 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
                                    12. 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
                                    13. Robinson BL et al: Magnetic resonance imaging of complex partial anomalous pulmonary venous return in adults. Circulation. 129(1):e1-2, 2014
                                    14. 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
                                    15. Seale AN et al: Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg. 145(5):1255-62, 2013
                                    16. 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
                                    17. 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
                                    18. 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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                                    Related Differential Diagnoses
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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

                                                                      1. Xiang M et al: Mixed type of total anomalous pulmonary venous connection: diagnosis, surgical approach and outcomes. J Cardiothorac Surg. 15(1):293, 2020
                                                                      2. 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
                                                                      3. 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
                                                                      4. 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
                                                                      5. White BR et al: Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction. Ann Thorac Surg. 108(1):122-9, 2019
                                                                      6. 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
                                                                      7. Tremblay C et al: Sutureless versus conventional pulmonary vein repair: a magnetic resonance pilot study. Ann Thorac Surg. 105(4):1248-54, 2018
                                                                      8. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                                                      9. 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
                                                                      10. Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
                                                                      11. 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
                                                                      12. 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
                                                                      13. Robinson BL et al: Magnetic resonance imaging of complex partial anomalous pulmonary venous return in adults. Circulation. 129(1):e1-2, 2014
                                                                      14. 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
                                                                      15. Seale AN et al: Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg. 145(5):1255-62, 2013
                                                                      16. 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
                                                                      17. 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
                                                                      18. Lakshminrusimha S et al: Use of CT angiography in the diagnosis of total anomalous venous return. J Perinatol. 29(6):458-61, 2009