link
Bookmarks
Ebstein Anomaly
Suhny Abbara, MD, FACR, FSCCT
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
7
0
4

KEY FACTS

  • Imaging

    • Top Differential Diagnoses

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Apical displacement of septal and posterior leaflets of tricuspid valve
              • Although annulus remains in normal position, proximal portions of leaflets are attached to ventricular walls, effectively displacing leaflet hingepoints toward apex
            • Classic plain film appearance: Massive right-sided cardiomegaly (box-shaped heart)
            • Category: Cyanotic, (severe) cardiomegaly, normal or decreased pulmonary vascularity
            • Hemodynamics: Severe tricuspid valve regurgitation
              • Volume overload to right heart
              • Right-to-left shunting through patent foramen ovale (PFO) leads to cyanosis

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Tobler D et al: Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol. 165(1):146-50, 2013
                                        2. Attenhofer Jost CH et al: Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging. 28(5):1147-59, 2012
                                        3. Beerepoot JP et al: Case 71: Ebstein anomaly. Radiology. 231(3):747-51, 2004
                                        4. Chauvaud S et al: Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 23(4):525-31, 2003
                                        5. Dearani JA et al: Ebstein's anomaly of the tricuspid valve. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 524-36, 2003
                                        6. MacLellan-Tobert SG et al: Ebstein anomaly of the tricuspid valve. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 461-8, 2000
                                        7. Ammash NM et al: Mimics of Ebstein's anomaly. Am Heart J. 134(3):508-13, 1997
                                        8. Choi YH et al: MR imaging of Ebstein's anomaly of the tricuspid valve. AJR Am J Roentgenol. 163(3):539-43, 1994
                                        9. Eustace S et al: Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis. Clin Radiol. 49(10):690-2, 1994
                                        10. Farb A et al: Anatomy and pathology of the right ventricle (including acquired tricuspid and pulmonic valve disease). Cardiol Clin. 10(1):1-21, 1992
                                        11. Lau MK et al: Magnetic resonance imaging of Ebstein's anomaly: report of two cases. J Formos Med Assoc. 91(12):1205-8, 1992
                                        12. Saxena A et al: Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging. J Am Coll Cardiol. 17(1):182-6, 1991
                                        13. Kastler B et al: Potential role of MR imaging in the diagnostic management of Ebstein anomaly in a newborn. J Comput Assist Tomogr. 14(5):825-7, 1990
                                        14. Link KM et al: MR imaging of Ebstein anomaly: results in four cases. AJR Am J Roentgenol. 150(2):363-7, 1988
                                        Related Anatomy
                                        Loading...
                                        Related Differential Diagnoses
                                        Loading...
                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Definitions

                                                  • Apical displacement of septal and posterior leaflets of tricuspid valve
                                                    • Although annulus remains in normal position, proximal portions of leaflets are attached to ventricular walls, effectively displacing leaflet hingepoints toward apex
                                                  • Classic plain film appearance: Massive right-sided cardiomegaly (box-shaped heart)
                                                  • Category: Cyanotic, (severe) cardiomegaly, normal or decreased pulmonary vascularity
                                                  • Hemodynamics: Severe tricuspid valve regurgitation
                                                    • Volume overload to right heart
                                                    • Right-to-left shunting through patent foramen ovale (PFO) leads to cyanosis

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Echocardiographic Findings

                                                          • Angiographic Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Tobler D et al: Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol. 165(1):146-50, 2013
                                                                              2. Attenhofer Jost CH et al: Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging. 28(5):1147-59, 2012
                                                                              3. Beerepoot JP et al: Case 71: Ebstein anomaly. Radiology. 231(3):747-51, 2004
                                                                              4. Chauvaud S et al: Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 23(4):525-31, 2003
                                                                              5. Dearani JA et al: Ebstein's anomaly of the tricuspid valve. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 524-36, 2003
                                                                              6. MacLellan-Tobert SG et al: Ebstein anomaly of the tricuspid valve. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 461-8, 2000
                                                                              7. Ammash NM et al: Mimics of Ebstein's anomaly. Am Heart J. 134(3):508-13, 1997
                                                                              8. Choi YH et al: MR imaging of Ebstein's anomaly of the tricuspid valve. AJR Am J Roentgenol. 163(3):539-43, 1994
                                                                              9. Eustace S et al: Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis. Clin Radiol. 49(10):690-2, 1994
                                                                              10. Farb A et al: Anatomy and pathology of the right ventricle (including acquired tricuspid and pulmonic valve disease). Cardiol Clin. 10(1):1-21, 1992
                                                                              11. Lau MK et al: Magnetic resonance imaging of Ebstein's anomaly: report of two cases. J Formos Med Assoc. 91(12):1205-8, 1992
                                                                              12. Saxena A et al: Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging. J Am Coll Cardiol. 17(1):182-6, 1991
                                                                              13. Kastler B et al: Potential role of MR imaging in the diagnostic management of Ebstein anomaly in a newborn. J Comput Assist Tomogr. 14(5):825-7, 1990
                                                                              14. Link KM et al: MR imaging of Ebstein anomaly: results in four cases. AJR Am J Roentgenol. 150(2):363-7, 1988