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Atrioventricular Septal Defect
Michael D. Puchalski, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Atrioventricular septal defect (AVSD)
          • Synonyms

            • Atrioventricular canal
            • Endocardial cushion defect
          • Definitions

            • Central cardiac defect involving
              • Atrial septum [atrial septal defect (ASD)]
              • Ventricular septum [ventricular septal defect (VSD)]
              • Atrioventricular (AV) valves
              • Abnormal course of conducting system
            • Defect can be balanced or unbalanced
              • Balanced: Right and left ventricles are equal in size with equal commitment of AV valves
              • Unbalanced: 1 ventricle gets majority of inflow and is equivalent to single ventricle
            • Defect can be complete or partial
              • Partial: Primum ASD, distinct mitral and tricuspid valve annuli, cleft in mitral valve
                • Transitional: Partial AVSD that also has small inlet VSD which is partially occluded by dense chordal attachments
              • Complete: Primum ASD, contiguous inlet VSD, and common AV valve has single annulus
                • Intermediate: Complete AVSD that has distinct right and left AV valve orifice despite having only 1 common annulus

          IMAGING

          • General Features

            • Echocardiographic Findings

              • Imaging Recommendations

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Staging, Grading, & Classification

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Ginde S et al: Long-term outcomes after surgical repair of complete atrioventricular septal defect. J Thorac Cardiovasc Surg. 150(2):369-74, 2015
                                  2. Gittenberger-de Groot AC et al: Morphogenesis and molecular considerations on congenital cardiac septal defects. Ann Med. 46(8):640-52, 2014
                                  3. Beppu H et al: BMP type II receptor regulates positioning of outflow tract and remodeling of atrioventricular cushion during cardiogenesis. Dev Biol. 331(2):167-75, 2009
                                  4. Shuhaiber JH et al: Current options and outcomes for the management of atrioventricular septal defect. Eur J Cardiothorac Surg. 35(5):891-900, 2009
                                  5. Goddeeris MM et al: Intracardiac septation requires hedgehog-dependent cellular contributions from outside the heart. Development. 135(10):1887-95, 2008
                                  6. Suzuki T et al: Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 86(2):596-602, 2008
                                  7. Fesslova V et al: Spectrum and outcome of atrioventricular septal defect in fetal life. Cardiol Young. 12(1):18-26, 2002
                                  8. Hoffman JI et al: The incidence of congenital heart disease. J Am Coll Cardiol. 39(12):1890-900, 2002
                                  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

                                            • Atrioventricular septal defect (AVSD)
                                          • Synonyms

                                            • Atrioventricular canal
                                            • Endocardial cushion defect
                                          • Definitions

                                            • Central cardiac defect involving
                                              • Atrial septum [atrial septal defect (ASD)]
                                              • Ventricular septum [ventricular septal defect (VSD)]
                                              • Atrioventricular (AV) valves
                                              • Abnormal course of conducting system
                                            • Defect can be balanced or unbalanced
                                              • Balanced: Right and left ventricles are equal in size with equal commitment of AV valves
                                              • Unbalanced: 1 ventricle gets majority of inflow and is equivalent to single ventricle
                                            • Defect can be complete or partial
                                              • Partial: Primum ASD, distinct mitral and tricuspid valve annuli, cleft in mitral valve
                                                • Transitional: Partial AVSD that also has small inlet VSD which is partially occluded by dense chordal attachments
                                              • Complete: Primum ASD, contiguous inlet VSD, and common AV valve has single annulus
                                                • Intermediate: Complete AVSD that has distinct right and left AV valve orifice despite having only 1 common annulus

                                          IMAGING

                                          • General Features

                                            • Echocardiographic Findings

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    • Staging, Grading, & Classification

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              DIAGNOSTIC CHECKLIST

                                                              • Consider

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Ginde S et al: Long-term outcomes after surgical repair of complete atrioventricular septal defect. J Thorac Cardiovasc Surg. 150(2):369-74, 2015
                                                                  2. Gittenberger-de Groot AC et al: Morphogenesis and molecular considerations on congenital cardiac septal defects. Ann Med. 46(8):640-52, 2014
                                                                  3. Beppu H et al: BMP type II receptor regulates positioning of outflow tract and remodeling of atrioventricular cushion during cardiogenesis. Dev Biol. 331(2):167-75, 2009
                                                                  4. Shuhaiber JH et al: Current options and outcomes for the management of atrioventricular septal defect. Eur J Cardiothorac Surg. 35(5):891-900, 2009
                                                                  5. Goddeeris MM et al: Intracardiac septation requires hedgehog-dependent cellular contributions from outside the heart. Development. 135(10):1887-95, 2008
                                                                  6. Suzuki T et al: Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 86(2):596-602, 2008
                                                                  7. Fesslova V et al: Spectrum and outcome of atrioventricular septal defect in fetal life. Cardiol Young. 12(1):18-26, 2002
                                                                  8. Hoffman JI et al: The incidence of congenital heart disease. J Am Coll Cardiol. 39(12):1890-900, 2002