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Endomyocardial Fibrosis
Jonathan D. Dodd, MD, MSc, MRCPI, FFR(RCSI); David Murphy, MD; Darragh Brady, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Endomyocardial fibrosis (EMF)
          • Synonyms

            • Davies disease
            • Restrictive obliterative cardiomyopathy
          • Definitions

            • Idiopathic disorder characterized by subendocardial fibrosis and restrictive cardiomyopathy
              • Endocardial fibrosis can affect all 4 cardiac chambers
              • This restrictive scarring prevents ventricular filling
              • Tethering of papillary muscles leads to valvular regurgitation
              • Most commonly affects right ventricle

          IMAGING

          • General Features

            • CT Findings

              • Imaging Recommendations

                • Radiographic Findings

                  • MR Findings

                    • Echocardiographic Findings

                      • Angiographic Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Gutierrez PS et al: Endomyocardial fibrosis. Autops Case Rep. 7(3):3-6, 2017
                                        2. Nogi M et al: Progressive mitral regurgitation in a patient with tropical endomyocardial fibrosis. Eur Heart J Cardiovasc Imaging. 18(9):1071, 2017
                                        3. Grimaldi A et al: Tropical endomyocardial fibrosis: natural history, challenges, and perspectives. Circulation. 133(24):2503-15, 2016
                                        4. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                        5. Dato I: How to recognize endomyocardial fibrosis? J Cardiovasc Med (Hagerstown). 16(8):547-51, 2015
                                        6. León D et al: Usefulness of cardiac MRI in the early diagnosis of endomyocardial fibrosis. Rev Port Cardiol. 31(5):401-2, 2012
                                        7. Mocumbi AO et al: Echocardiography accurately assesses the pathological abnormalities of chronic endomyocardial fibrosis. Int J Cardiovasc Imaging. 27(7):955-64, 2011
                                        8. Qureshi N et al: MR imaging of endomyocardial fibrosis (EMF). Int J Cardiol. 149(1):e36-7, 2011
                                        9. Salemi VM et al: Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients. Circ Cardiovasc Imaging. 4(3):304-11, 2011
                                        10. Marijon E et al: Typical clinical aspect of endomyocardial fibrosis. Int J Cardiol. 112(2):259-60, 2006
                                        11. Hassan WM et al: Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest. 128(6):3985-92, 2005
                                        12. DAVIES JN: Endomyocardial fibrosis in Uganda. Cent Afr J Med. 2(9):323-8, 1956
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Endomyocardial fibrosis (EMF)
                                                • Synonyms

                                                  • Davies disease
                                                  • Restrictive obliterative cardiomyopathy
                                                • Definitions

                                                  • Idiopathic disorder characterized by subendocardial fibrosis and restrictive cardiomyopathy
                                                    • Endocardial fibrosis can affect all 4 cardiac chambers
                                                    • This restrictive scarring prevents ventricular filling
                                                    • Tethering of papillary muscles leads to valvular regurgitation
                                                    • Most commonly affects right ventricle

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • Imaging Recommendations

                                                      • Radiographic Findings

                                                        • MR Findings

                                                          • Echocardiographic Findings

                                                            • Angiographic Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Gutierrez PS et al: Endomyocardial fibrosis. Autops Case Rep. 7(3):3-6, 2017
                                                                              2. Nogi M et al: Progressive mitral regurgitation in a patient with tropical endomyocardial fibrosis. Eur Heart J Cardiovasc Imaging. 18(9):1071, 2017
                                                                              3. Grimaldi A et al: Tropical endomyocardial fibrosis: natural history, challenges, and perspectives. Circulation. 133(24):2503-15, 2016
                                                                              4. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                                                              5. Dato I: How to recognize endomyocardial fibrosis? J Cardiovasc Med (Hagerstown). 16(8):547-51, 2015
                                                                              6. León D et al: Usefulness of cardiac MRI in the early diagnosis of endomyocardial fibrosis. Rev Port Cardiol. 31(5):401-2, 2012
                                                                              7. Mocumbi AO et al: Echocardiography accurately assesses the pathological abnormalities of chronic endomyocardial fibrosis. Int J Cardiovasc Imaging. 27(7):955-64, 2011
                                                                              8. Qureshi N et al: MR imaging of endomyocardial fibrosis (EMF). Int J Cardiol. 149(1):e36-7, 2011
                                                                              9. Salemi VM et al: Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients. Circ Cardiovasc Imaging. 4(3):304-11, 2011
                                                                              10. Marijon E et al: Typical clinical aspect of endomyocardial fibrosis. Int J Cardiol. 112(2):259-60, 2006
                                                                              11. Hassan WM et al: Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest. 128(6):3985-92, 2005
                                                                              12. DAVIES JN: Endomyocardial fibrosis in Uganda. Cent Afr J Med. 2(9):323-8, 1956