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Pediatrics
Diagnosis
Cardiac
Congenital Heart Disease
Ebstein Anomaly
Ebstein Anomaly
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Downward/apical displacement of septal & posterior leaflets of tricuspid valve → tricuspid regurgitation
            • Classic radiographic appearance: Massive right-sided cardiomegaly (box-shaped heart)
            • Category: Cyanotic, (severe) cardiomegaly, normal or ↓ pulmonary vascularity
            • Hemodynamics: Determined by severity of tricuspid valve regurgitation
              • Volume overload to right heart
              • Right-to-left shunting through patent foramen ovale (PFO) → cyanosis

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Qureshi MY et al: Commentary: gold or silver? Value of cardiac magnetic resonance imaging over echocardiography in Ebstein's anomaly. J Thorac Cardiovasc Surg. 161(3):1109-10, 2021
                                      2. Rajagopal R et al: Ebstein's anomaly with imperforate tricuspid valve: an extremely rare congenital anomaly. J Cardiovasc Comput Tomogr. 14(6):e95-6, 2020
                                      3. Holst KA et al: Ebstein's anomaly. Methodist Debakey Cardiovasc J. 15(2):138-44, 2019
                                      4. Mrad Agua K et al: Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly. Eur J Cardiothorac Surg. 55(6):1187-93, 2019
                                      5. Qureshi MY et al: Tricuspid valve imaging and intervention in pediatric and adult patients with congenital heart disease. JACC Cardiovasc Imaging. 12(4):637-51, 2019
                                      6. Ciepłucha A et al: Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly. Heart Vessels. 33(9):1076-85, 2018
                                      7. Qureshi MY et al: Cardiac imaging in Ebstein anomaly. Trends Cardiovasc Med. 28(6):403-9, 2018
                                      8. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                      9. Kumar P et al: Ebstein anomaly with right atrial clot. Cardiol Res. 6(4-5):319-23, 2015
                                      10. Kühn A et al: Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging. 17(8):930-5, 2016
                                      11. Li X et al: More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol. 206:131-7, 2016
                                      12. Malik SB et al: The right atrium: gateway to the heart--anatomic and pathologic imaging findings. Radiographics. 35(1):14-31, 2015
                                      13. Arya P et al: Ebstein anomaly: assessment, management, and timing of intervention. Curr Treat Options Cardiovasc Med. 16(10):338, 2014
                                      14. Hösch O et al: The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities. Circ Cardiovasc Imaging. 7(4):601-9, 2014
                                      15. Bonello B et al: Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 105(11):605-13, 2012
                                      16. Zikria JF et al: Common CTA features of Ebstein anomaly in a middle-aged woman with a heart murmur and dyspnea on exertion. J Cardiovasc Comput Tomogr. 6(6):431-2, 2012
                                      17. Yalonetsky S et al: Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults. Am J Cardiol. 107(5):767-73, 2011
                                      18. Nakamura I et al: Ebstein anomaly by cardiac magnetic resonance imaging. J Am Coll Cardiol. 53(17):1568, 2009
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Definitions

                                                • Downward/apical displacement of septal & posterior leaflets of tricuspid valve → tricuspid regurgitation
                                                • Classic radiographic appearance: Massive right-sided cardiomegaly (box-shaped heart)
                                                • Category: Cyanotic, (severe) cardiomegaly, normal or ↓ pulmonary vascularity
                                                • Hemodynamics: Determined by severity of tricuspid valve regurgitation
                                                  • Volume overload to right heart
                                                  • Right-to-left shunting through patent foramen ovale (PFO) → cyanosis

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Echocardiographic Findings

                                                        • Angiographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Qureshi MY et al: Commentary: gold or silver? Value of cardiac magnetic resonance imaging over echocardiography in Ebstein's anomaly. J Thorac Cardiovasc Surg. 161(3):1109-10, 2021
                                                                          2. Rajagopal R et al: Ebstein's anomaly with imperforate tricuspid valve: an extremely rare congenital anomaly. J Cardiovasc Comput Tomogr. 14(6):e95-6, 2020
                                                                          3. Holst KA et al: Ebstein's anomaly. Methodist Debakey Cardiovasc J. 15(2):138-44, 2019
                                                                          4. Mrad Agua K et al: Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly. Eur J Cardiothorac Surg. 55(6):1187-93, 2019
                                                                          5. Qureshi MY et al: Tricuspid valve imaging and intervention in pediatric and adult patients with congenital heart disease. JACC Cardiovasc Imaging. 12(4):637-51, 2019
                                                                          6. Ciepłucha A et al: Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly. Heart Vessels. 33(9):1076-85, 2018
                                                                          7. Qureshi MY et al: Cardiac imaging in Ebstein anomaly. Trends Cardiovasc Med. 28(6):403-9, 2018
                                                                          8. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                                                          9. Kumar P et al: Ebstein anomaly with right atrial clot. Cardiol Res. 6(4-5):319-23, 2015
                                                                          10. Kühn A et al: Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging. 17(8):930-5, 2016
                                                                          11. Li X et al: More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol. 206:131-7, 2016
                                                                          12. Malik SB et al: The right atrium: gateway to the heart--anatomic and pathologic imaging findings. Radiographics. 35(1):14-31, 2015
                                                                          13. Arya P et al: Ebstein anomaly: assessment, management, and timing of intervention. Curr Treat Options Cardiovasc Med. 16(10):338, 2014
                                                                          14. Hösch O et al: The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities. Circ Cardiovasc Imaging. 7(4):601-9, 2014
                                                                          15. Bonello B et al: Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 105(11):605-13, 2012
                                                                          16. Zikria JF et al: Common CTA features of Ebstein anomaly in a middle-aged woman with a heart murmur and dyspnea on exertion. J Cardiovasc Comput Tomogr. 6(6):431-2, 2012
                                                                          17. Yalonetsky S et al: Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults. Am J Cardiol. 107(5):767-73, 2011
                                                                          18. Nakamura I et al: Ebstein anomaly by cardiac magnetic resonance imaging. J Am Coll Cardiol. 53(17):1568, 2009