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Total Anomalous Pulmonary Venous Return
Michael D. Puchalski, MD; Anne M. Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Total anomalous pulmonary venous return (TAPVR)
            • Partial anomalous pulmonary venous return (PAPVR)
            • Total (TAPVC) and partial pulmonary venous connection (PAPVC)
              • 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 (PV) connect to systemic veins, right atrium (RA) or both
              • 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
                  • For example, 2 right veins may drain to supracardiac location and 2 left veins may drain below diaphragm
            • 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

                                  1. Koneti NR et al: Surgical correction of obstructed total anomalous pulmonary venous return soon after birth. Ann Pediatr Cardiol. 8(3):255-6, 2015
                                  2. 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
                                  3. 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
                                  4. Laux D et al: Prenatal diagnosis of isolated total anomalous pulmonary venous connection: a series of 10 cases. Ultrasound Obstet Gynecol. 41(3):291-7, 2013
                                  5. Sinkovskaya E et al: A novel systematic approach to the evaluation of the fetal venous system. Semin Fetal Neonatal Med. 18(5):269-78, 2013
                                  6. Pinto NM et al: Barriers to prenatal detection of congenital heart disease: a population-based study. Ultrasound Obstet Gynecol. 40(4):418-25, 2012
                                  7. Seale AN et al: Total anomalous pulmonary venous connection: impact of prenatal diagnosis. Ultrasound Obstet Gynecol. 40(3):310-8, 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)
                                            • Partial anomalous pulmonary venous return (PAPVR)
                                            • Total (TAPVC) and partial pulmonary venous connection (PAPVC)
                                              • 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 (PV) connect to systemic veins, right atrium (RA) or both
                                              • 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
                                                  • For example, 2 right veins may drain to supracardiac location and 2 left veins may drain below diaphragm
                                            • 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

                                                                  1. Koneti NR et al: Surgical correction of obstructed total anomalous pulmonary venous return soon after birth. Ann Pediatr Cardiol. 8(3):255-6, 2015
                                                                  2. 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
                                                                  3. 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
                                                                  4. Laux D et al: Prenatal diagnosis of isolated total anomalous pulmonary venous connection: a series of 10 cases. Ultrasound Obstet Gynecol. 41(3):291-7, 2013
                                                                  5. Sinkovskaya E et al: A novel systematic approach to the evaluation of the fetal venous system. Semin Fetal Neonatal Med. 18(5):269-78, 2013
                                                                  6. Pinto NM et al: Barriers to prenatal detection of congenital heart disease: a population-based study. Ultrasound Obstet Gynecol. 40(4):418-25, 2012
                                                                  7. Seale AN et al: Total anomalous pulmonary venous connection: impact of prenatal diagnosis. Ultrasound Obstet Gynecol. 40(3):310-8, 2012