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Total Anomalous Pulmonary Venous Return
Jonathan Hero Chung, MD; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          TERMINOLOGY

          • Abbreviations

            • Total anomalous pulmonary venous return (TAPVR) or connection (TAPVC)
          • Synonyms

            • Total anomalous pulmonary venous connection
          • Definitions

            • Embryologic failure of common pulmonary vein (PV) to connect to left atrium (LA)
              • Anomalous connection of PVs to right atrium (RA), coronary sinus, systemic veins, or tributaries leading to left-to-right shunt
              • Atrial septal defect (ASD) of varying size (right-to-left shunt) resulting in admixture lesion

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Echocardiographic Findings

                      • Angiographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            Selected References

                                            1. T├╝rkvatan A et al: Multidetector computed tomographic angiography imaging of congenital pulmonary venous anomalies: a pictorial review. Can Assoc Radiol J. 68(1):66-76, 2017
                                            2. Husain SA et al: Total anomalous pulmonary venous connection: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg. 94(3):825-31; discussion 831-2, 2012
                                            3. Molinari F et al: Total anomalous pulmonary venous return with connection to the supradiaphragmatic inferior vena cava: assessment and diagnosis by multidetector computed tomography. Heart Lung Circ. 20(5):341-2, 2011
                                            4. Seale AN et al: Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 122(25):2718-26, 2010
                                            5. Lakshminrusimha S et al: Use of CT angiography in the diagnosis of total anomalous venous return. J Perinatol. 29(6):458-61, 2009
                                            6. Oh KH et al: Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography. Pediatr Radiol. 39(9):950-4, 2009
                                            7. Vavas E et al: Total anomalous pulmonary venous connection in an adult: comprehensive multimodality evaluation. Congenit Heart Dis. 4(5):384-6, 2009
                                            8. Ferguson EC et al: Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 27(5):1323-34, 2007
                                            9. Gallego C et al: Congenital hepatic shunts. Radiographics. 24(3):755-72, 2004
                                            10. Chen SJ et al: Validation of pulmonary venous obstruction by electron beam computed tomography in children with congenital heart disease. Am J Cardiol. 87(5):589-93, 2001
                                            11. Videlefsky N et al: Magnetic resonance phase-shift velocity mapping in pediatric patients with pulmonary venous obstruction. J Am Coll Cardiol. 38(1):262-7, 2001
                                            12. Kim TH et al: Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol. 175(5):1381-6, 2000
                                            13. Livolsi A et al: MR diagnosis of subdiaphragmatic anomalous pulmonary venous drainage in a newborn. J Comput Assist Tomogr. 15(6):1051-3, 1991
                                            14. Duff DF et al: Infradiaphragmatic total anomalous pulmonary venous return. Review of clinical and pathological findings and results of operation in 28 cases. Br Heart J. 39(6):619-26, 1977
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Total anomalous pulmonary venous return (TAPVR) or connection (TAPVC)
                                                    • Synonyms

                                                      • Total anomalous pulmonary venous connection
                                                    • Definitions

                                                      • Embryologic failure of common pulmonary vein (PV) to connect to left atrium (LA)
                                                        • Anomalous connection of PVs to right atrium (RA), coronary sinus, systemic veins, or tributaries leading to left-to-right shunt
                                                        • Atrial septal defect (ASD) of varying size (right-to-left shunt) resulting in admixture lesion

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Ultrasonographic Findings

                                                              • Echocardiographic Findings

                                                                • Angiographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Consider

                                                                                      Selected References

                                                                                      1. T├╝rkvatan A et al: Multidetector computed tomographic angiography imaging of congenital pulmonary venous anomalies: a pictorial review. Can Assoc Radiol J. 68(1):66-76, 2017
                                                                                      2. Husain SA et al: Total anomalous pulmonary venous connection: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg. 94(3):825-31; discussion 831-2, 2012
                                                                                      3. Molinari F et al: Total anomalous pulmonary venous return with connection to the supradiaphragmatic inferior vena cava: assessment and diagnosis by multidetector computed tomography. Heart Lung Circ. 20(5):341-2, 2011
                                                                                      4. Seale AN et al: Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 122(25):2718-26, 2010
                                                                                      5. Lakshminrusimha S et al: Use of CT angiography in the diagnosis of total anomalous venous return. J Perinatol. 29(6):458-61, 2009
                                                                                      6. Oh KH et al: Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography. Pediatr Radiol. 39(9):950-4, 2009
                                                                                      7. Vavas E et al: Total anomalous pulmonary venous connection in an adult: comprehensive multimodality evaluation. Congenit Heart Dis. 4(5):384-6, 2009
                                                                                      8. Ferguson EC et al: Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 27(5):1323-34, 2007
                                                                                      9. Gallego C et al: Congenital hepatic shunts. Radiographics. 24(3):755-72, 2004
                                                                                      10. Chen SJ et al: Validation of pulmonary venous obstruction by electron beam computed tomography in children with congenital heart disease. Am J Cardiol. 87(5):589-93, 2001
                                                                                      11. Videlefsky N et al: Magnetic resonance phase-shift velocity mapping in pediatric patients with pulmonary venous obstruction. J Am Coll Cardiol. 38(1):262-7, 2001
                                                                                      12. Kim TH et al: Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol. 175(5):1381-6, 2000
                                                                                      13. Livolsi A et al: MR diagnosis of subdiaphragmatic anomalous pulmonary venous drainage in a newborn. J Comput Assist Tomogr. 15(6):1051-3, 1991
                                                                                      14. Duff DF et al: Infradiaphragmatic total anomalous pulmonary venous return. Review of clinical and pathological findings and results of operation in 28 cases. Br Heart J. 39(6):619-26, 1977