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Pediatrics
Diagnosis
Cardiac
Congenital Heart Disease
Atrioventricular Septal Defect
Atrioventricular Septal Defect
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Atrioventricular septal defect (AVSD)

        • Synonyms

          • AVSD
            • Atrioventricular canal (AVC) defect
            • Endocardial cushion defect
            • Complete AVC defect
          • Ostium primum defect
            • Partial AVC defect
            • Partial AVSD
            • Incomplete endocardial cushion defect

        • Definitions

          • Broad spectrum of defects characterized by involvement of atrial septum, ventricular septum, & 1 or both AV valves
            • Complete AVSD indicates presence of both atrial & ventricular septal defects with common AV valve
            • Partial AVSD indicates atrial septal involvement with separate mitral & tricuspid valve orifices
            • Unbalanced AVSD indicates 1 ventricular chamber is hypoplastic as compared to other, depending on direction of AV valve flow

        IMAGING

        • General Features

          • Echocardiographic Findings

            • CT Findings

              • MR Findings

                • Angiographic Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Nayak S et al: Echocardiographic assessment of atrioventricular canal defects. Echocardiography. 37(12):2199-210, 2020
                                2. Elders B et al: Altered ascending aortic wall shear stress in patients with corrected atrioventricular septal defect: a comprehensive cardiovascular magnetic resonance and 4D flow MRI evaluation. Cardiol Young. 29(5):637-42, 2019
                                3. Ye XT et al: Partition of common atrioventricular valve in a patient with dextrocardia and univentricular circulation. Semin Thorac Cardiovasc Surg. 31(1):113-5, 2019
                                4. Han J et al: Goose neck appearance: endocardial cushion defect. J Med Imaging Radiat Oncol. 62 Suppl 1:31, 2018
                                5. Sarısoy Ö et al: Long-term outcomes in patients who underwent surgical correction for atrioventricular septal defect. Anatol J Cardiol. 20(4):229-34, 2018
                                6. Zhu Y et al: Preliminary study of the application of transthoracic echocardiography-guided three-dimensional printing for the assessment of structural heart disease. Echocardiography. 34(12):1903-8, 2017
                                7. Davey BT et al: The natural history of atrioventricular valve regurgitation throughout fetal life in patients with atrioventricular canal defects. Pediatr Cardiol. 37(1):50-4, 2016
                                8. Saremi F et al: Septal atrioventricular junction region: comprehensive imaging in adults. Radiographics. 36(7):1966-86, 2016
                                9. Calkoen EE et al: Characterization and quantification of dynamic eccentric regurgitation of the left atrioventricular valve after atrioventricular septal defect correction with 4D flow cardiovascular magnetic resonance and retrospective valve tracking. J Cardiovasc Magn Reson. 17:18, 2015
                                10. Rice K et al: Three-dimensional echocardiography of congenital abnormalities of the left atrioventricular valve. Echo Res Pract. 2(1):R13-24, 2015
                                11. Salizzoni S et al: An iatrogenic atrioventricular septal defect that developed following transfemoral TAVI. J Heart Valve Dis. 23(2):216-8, 2014
                                12. Richardson, RR: Atlas of Pediatric Cardiac CTA Congenital Heart Disease. New York, NY: Springer, 2013
                                13. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                14. Bell A et al: Noninvasive assessment of pulmonary artery flow and resistance by cardiac magnetic resonance in congenital heart diseases with unrestricted left-to-right shunt. JACC Cardiovasc Imaging. 2(11):1285-91, 2009
                                15. Nicol ED et al: Assessment of adult congenital heart disease with multi-detector computed tomography: beyond coronary lumenography. Clin Radiol. 62(6):518-27, 2007
                                16. Ten Harkel AD et al: Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg. 79(2):607-12, 2005
                                17. Formigari R et al: Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. 78(2):666-72; discussion 672, 2004
                                18. Freeman SB et al: Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 80(3):213-7, 1998
                                19. van Son JA et al: Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. Ann Thorac Surg. 63(6):1657-63, 1997
                                20. Tweddell JS et al: Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. 62(2):419-24, 1996
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Clinical Issues

                                      TERMINOLOGY

                                      • Abbreviations

                                        • Atrioventricular septal defect (AVSD)

                                      • Synonyms

                                        • AVSD
                                          • Atrioventricular canal (AVC) defect
                                          • Endocardial cushion defect
                                          • Complete AVC defect
                                        • Ostium primum defect
                                          • Partial AVC defect
                                          • Partial AVSD
                                          • Incomplete endocardial cushion defect

                                      • Definitions

                                        • Broad spectrum of defects characterized by involvement of atrial septum, ventricular septum, & 1 or both AV valves
                                          • Complete AVSD indicates presence of both atrial & ventricular septal defects with common AV valve
                                          • Partial AVSD indicates atrial septal involvement with separate mitral & tricuspid valve orifices
                                          • Unbalanced AVSD indicates 1 ventricular chamber is hypoplastic as compared to other, depending on direction of AV valve flow

                                      IMAGING

                                      • General Features

                                        • Echocardiographic Findings

                                          • CT Findings

                                            • MR Findings

                                              • Angiographic Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Nayak S et al: Echocardiographic assessment of atrioventricular canal defects. Echocardiography. 37(12):2199-210, 2020
                                                              2. Elders B et al: Altered ascending aortic wall shear stress in patients with corrected atrioventricular septal defect: a comprehensive cardiovascular magnetic resonance and 4D flow MRI evaluation. Cardiol Young. 29(5):637-42, 2019
                                                              3. Ye XT et al: Partition of common atrioventricular valve in a patient with dextrocardia and univentricular circulation. Semin Thorac Cardiovasc Surg. 31(1):113-5, 2019
                                                              4. Han J et al: Goose neck appearance: endocardial cushion defect. J Med Imaging Radiat Oncol. 62 Suppl 1:31, 2018
                                                              5. Sarısoy Ö et al: Long-term outcomes in patients who underwent surgical correction for atrioventricular septal defect. Anatol J Cardiol. 20(4):229-34, 2018
                                                              6. Zhu Y et al: Preliminary study of the application of transthoracic echocardiography-guided three-dimensional printing for the assessment of structural heart disease. Echocardiography. 34(12):1903-8, 2017
                                                              7. Davey BT et al: The natural history of atrioventricular valve regurgitation throughout fetal life in patients with atrioventricular canal defects. Pediatr Cardiol. 37(1):50-4, 2016
                                                              8. Saremi F et al: Septal atrioventricular junction region: comprehensive imaging in adults. Radiographics. 36(7):1966-86, 2016
                                                              9. Calkoen EE et al: Characterization and quantification of dynamic eccentric regurgitation of the left atrioventricular valve after atrioventricular septal defect correction with 4D flow cardiovascular magnetic resonance and retrospective valve tracking. J Cardiovasc Magn Reson. 17:18, 2015
                                                              10. Rice K et al: Three-dimensional echocardiography of congenital abnormalities of the left atrioventricular valve. Echo Res Pract. 2(1):R13-24, 2015
                                                              11. Salizzoni S et al: An iatrogenic atrioventricular septal defect that developed following transfemoral TAVI. J Heart Valve Dis. 23(2):216-8, 2014
                                                              12. Richardson, RR: Atlas of Pediatric Cardiac CTA Congenital Heart Disease. New York, NY: Springer, 2013
                                                              13. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                                              14. Bell A et al: Noninvasive assessment of pulmonary artery flow and resistance by cardiac magnetic resonance in congenital heart diseases with unrestricted left-to-right shunt. JACC Cardiovasc Imaging. 2(11):1285-91, 2009
                                                              15. Nicol ED et al: Assessment of adult congenital heart disease with multi-detector computed tomography: beyond coronary lumenography. Clin Radiol. 62(6):518-27, 2007
                                                              16. Ten Harkel AD et al: Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg. 79(2):607-12, 2005
                                                              17. Formigari R et al: Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. 78(2):666-72; discussion 672, 2004
                                                              18. Freeman SB et al: Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 80(3):213-7, 1998
                                                              19. van Son JA et al: Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. Ann Thorac Surg. 63(6):1657-63, 1997
                                                              20. Tweddell JS et al: Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. 62(2):419-24, 1996