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Ebstein Anomaly
Whitnee Hogan, MD; Michael D. Puchalski, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Definitions

            • Apical displacement of septal and posterior tricuspid valve (TV) leaflets
              • Coaptation point of TV is lowered into right ventricle (RV), not at atrioventricular junction
                • Results in "atrialization" of RV

          IMAGING

          • General Features

            • Echocardiographic Findings

              • Imaging Recommendations

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    CLINICAL ISSUES

                    • Presentation

                      • Demographics

                        • Natural History & Prognosis

                          • Treatment

                            DIAGNOSTIC CHECKLIST

                            • Image Interpretation Pearls

                              Selected References

                              1. Freud LR et al: Risk factors for mortality and circulatory outcome among neonates prenatally diagnosed with Ebstein anomaly or tricuspid valve dysplasia: a multicenter study. J Am Heart Assoc. 9(21):e016684, 2020
                              2. Masoller N et al: Prediction of perinatal mortality in Ebstein's anomaly diagnosed in the second trimester of pregnancy. Fetal Diagn Ther. 47(8):604-14, 2020
                              3. Rydzewska K et al: Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management. J Matern Fetal Neonatal Med. 1-8, 2020
                              4. Wang S et al: Extracardiac Doppler indices predict perinatal mortality in fetuses with Ebstein anomaly and tricuspid valve dysplasia. Prenat Diagn. ePub, 2020
                              5. Selamet Tierney ES et al: Assessment of progressive pathophysiology after early prenatal diagnosis of the Ebstein anomaly or tricuspid valve dysplasia. Am J Cardiol. 119(1):106-11, 2017
                              6. Freud LR et al: Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation. 132(6):481-9, 2015
                              7. Ibrahim M et al: Cone reconstruction for Ebstein's anomaly: patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg. 149(4):1144-50, 2015
                              8. Luu Q et al: Ebstein's anomaly in those surviving to adult life - a single centre experience. Heart Lung Circ. 24(10):996-1001, 2015
                              9. Freire G et al: Impact of prenatal haemodynamic and functional abnormalities in Ebstein's anomaly on survival. Cardiol Young. 24(6):1049-56, 2014
                              10. Shinkawa T et al: Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg. 139(2):354-8, 2010
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Imaging

                                  • Clinical Issues

                                    • Diagnostic Checklist

                                      TERMINOLOGY

                                      • Definitions

                                        • Apical displacement of septal and posterior tricuspid valve (TV) leaflets
                                          • Coaptation point of TV is lowered into right ventricle (RV), not at atrioventricular junction
                                            • Results in "atrialization" of RV

                                      IMAGING

                                      • General Features

                                        • Echocardiographic Findings

                                          • Imaging Recommendations

                                            DIFFERENTIAL DIAGNOSIS

                                              PATHOLOGY

                                              • General Features

                                                CLINICAL ISSUES

                                                • Presentation

                                                  • Demographics

                                                    • Natural History & Prognosis

                                                      • Treatment

                                                        DIAGNOSTIC CHECKLIST

                                                        • Image Interpretation Pearls

                                                          Selected References

                                                          1. Freud LR et al: Risk factors for mortality and circulatory outcome among neonates prenatally diagnosed with Ebstein anomaly or tricuspid valve dysplasia: a multicenter study. J Am Heart Assoc. 9(21):e016684, 2020
                                                          2. Masoller N et al: Prediction of perinatal mortality in Ebstein's anomaly diagnosed in the second trimester of pregnancy. Fetal Diagn Ther. 47(8):604-14, 2020
                                                          3. Rydzewska K et al: Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management. J Matern Fetal Neonatal Med. 1-8, 2020
                                                          4. Wang S et al: Extracardiac Doppler indices predict perinatal mortality in fetuses with Ebstein anomaly and tricuspid valve dysplasia. Prenat Diagn. ePub, 2020
                                                          5. Selamet Tierney ES et al: Assessment of progressive pathophysiology after early prenatal diagnosis of the Ebstein anomaly or tricuspid valve dysplasia. Am J Cardiol. 119(1):106-11, 2017
                                                          6. Freud LR et al: Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation. 132(6):481-9, 2015
                                                          7. Ibrahim M et al: Cone reconstruction for Ebstein's anomaly: patient outcomes, biventricular function, and cardiopulmonary exercise capacity. J Thorac Cardiovasc Surg. 149(4):1144-50, 2015
                                                          8. Luu Q et al: Ebstein's anomaly in those surviving to adult life - a single centre experience. Heart Lung Circ. 24(10):996-1001, 2015
                                                          9. Freire G et al: Impact of prenatal haemodynamic and functional abnormalities in Ebstein's anomaly on survival. Cardiol Young. 24(6):1049-56, 2014
                                                          10. Shinkawa T et al: Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg. 139(2):354-8, 2010