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Cardiomyopathies
Duchenne Muscular Dystrophy-Related Cardiomyopathy
Duchenne Muscular Dystrophy-Related Cardiomyopathy
Randy R. Richardson, MD; Ryan A. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Duchenne muscular dystrophy (DMD)

            • Definitions

              • Dystrophin-associated inherited disorders with progressive muscle wasting & weakness

            IMAGING

            • General Features

              • Echocardiographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      • Reporting Tips

                                        Selected References

                                        1. Lee S et al: The role of imaging in characterizing the cardiac natural history of Duchenne muscular dystrophy. Pediatr Pulmonol. 56(4):766-81, 2021
                                        2. Sanchez F et al: Cardiac MR imaging of muscular dystrophies. Curr Probl Diagn Radiol. ePub, 2021
                                        3. Shih JA et al: Duchenne muscular dystrophy: the heart of the matter. Curr Heart Fail Rep. 17(3):57-66, 2020
                                        4. Hor KN et al: Advances in the diagnosis and management of cardiomyopathy in Duchenne muscular dystrophy. Neuromuscul Disord. 28(9):711-6, 2018
                                        5. Power LC et al: Imaging the heart to detect cardiomyopathy in Duchenne muscular dystrophy: a review. Neuromuscul Disord. 28(9):717-30, 2018
                                        6. D'Amario D et al: A current approach to heart failure in Duchenne muscular dystrophy. Heart. 103(22):1770-9, 2017
                                        7. Soslow JH et al: Increased myocardial native T1 and extracellular volume in patients with Duchenne muscular dystrophy. J Cardiovasc Magn Reson. 18:5, 2016
                                        8. Lang SM et al: Myocardial fibrosis and left ventricular dysfunction in Duchenne muscular dystrophy carriers using cardiac magnetic resonance imaging. Pediatr Cardiol. 36(7):1495-501, 2015
                                        9. Tandon A et al: Dystrophin genotype-cardiac phenotype correlations in Duchenne and Becker muscular dystrophies using cardiac magnetic resonance imaging. Am J Cardiol. 115(7):967-71, 2015
                                        10. Tandon A et al: Myocardial fibrosis burden predicts left ventricular ejection fraction and is associated with age and steroid treatment duration in duchenne muscular dystrophy. J Am Heart Assoc. 4(4), 2015
                                        11. Mazur W et al: Patterns of left ventricular remodeling in patients with Duchenne muscular dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain. Int J Cardiovasc Imaging. 28(1):99-107, 2011
                                        12. Verhaert D et al: Cardiac involvement in patients with muscular dystrophies: magnetic resonance imaging phenotype and genotypic considerations. Circ Cardiovasc Imaging. 4(1):67-76, 2011
                                        13. Hagenbuch SC et al: Detection of progressive cardiac dysfunction by serial evaluation of circumferential strain in patients with Duchenne muscular dystrophy. Am J Cardiol. 105(10):1451-5, 2010
                                        14. Hor KN et al: Comparison of magnetic resonance feature tracking for strain calculation with harmonic phase imaging analysis. JACC Cardiovasc Imaging. 3(2):144-51, 2010
                                        15. Hor KN et al: Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study. J Am Coll Cardiol. 53(14):1204-10, 2009
                                        16. Puchalski MD et al: Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy? Int J Cardiovasc Imaging. 25(1):57-63, 2009
                                        17. Rhodes J et al: Safety and efficacy of carvedilol therapy for patients with dilated cardiomyopathy secondary to muscular dystrophy. Pediatr Cardiol. 29(2):343-51, 2008
                                        18. Silva MC et al: Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy. J Am Coll Cardiol. 49(18):1874-9, 2007
                                        19. Ashford MW Jr et al: Occult cardiac contractile dysfunction in dystrophin-deficient children revealed by cardiac magnetic resonance strain imaging. Circulation. 112(16):2462-7, 2005
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Duchenne muscular dystrophy (DMD)

                                                  • Definitions

                                                    • Dystrophin-associated inherited disorders with progressive muscle wasting & weakness

                                                  IMAGING

                                                  • General Features

                                                    • Echocardiographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          • Image Interpretation Pearls

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Lee S et al: The role of imaging in characterizing the cardiac natural history of Duchenne muscular dystrophy. Pediatr Pulmonol. 56(4):766-81, 2021
                                                                              2. Sanchez F et al: Cardiac MR imaging of muscular dystrophies. Curr Probl Diagn Radiol. ePub, 2021
                                                                              3. Shih JA et al: Duchenne muscular dystrophy: the heart of the matter. Curr Heart Fail Rep. 17(3):57-66, 2020
                                                                              4. Hor KN et al: Advances in the diagnosis and management of cardiomyopathy in Duchenne muscular dystrophy. Neuromuscul Disord. 28(9):711-6, 2018
                                                                              5. Power LC et al: Imaging the heart to detect cardiomyopathy in Duchenne muscular dystrophy: a review. Neuromuscul Disord. 28(9):717-30, 2018
                                                                              6. D'Amario D et al: A current approach to heart failure in Duchenne muscular dystrophy. Heart. 103(22):1770-9, 2017
                                                                              7. Soslow JH et al: Increased myocardial native T1 and extracellular volume in patients with Duchenne muscular dystrophy. J Cardiovasc Magn Reson. 18:5, 2016
                                                                              8. Lang SM et al: Myocardial fibrosis and left ventricular dysfunction in Duchenne muscular dystrophy carriers using cardiac magnetic resonance imaging. Pediatr Cardiol. 36(7):1495-501, 2015
                                                                              9. Tandon A et al: Dystrophin genotype-cardiac phenotype correlations in Duchenne and Becker muscular dystrophies using cardiac magnetic resonance imaging. Am J Cardiol. 115(7):967-71, 2015
                                                                              10. Tandon A et al: Myocardial fibrosis burden predicts left ventricular ejection fraction and is associated with age and steroid treatment duration in duchenne muscular dystrophy. J Am Heart Assoc. 4(4), 2015
                                                                              11. Mazur W et al: Patterns of left ventricular remodeling in patients with Duchenne muscular dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain. Int J Cardiovasc Imaging. 28(1):99-107, 2011
                                                                              12. Verhaert D et al: Cardiac involvement in patients with muscular dystrophies: magnetic resonance imaging phenotype and genotypic considerations. Circ Cardiovasc Imaging. 4(1):67-76, 2011
                                                                              13. Hagenbuch SC et al: Detection of progressive cardiac dysfunction by serial evaluation of circumferential strain in patients with Duchenne muscular dystrophy. Am J Cardiol. 105(10):1451-5, 2010
                                                                              14. Hor KN et al: Comparison of magnetic resonance feature tracking for strain calculation with harmonic phase imaging analysis. JACC Cardiovasc Imaging. 3(2):144-51, 2010
                                                                              15. Hor KN et al: Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study. J Am Coll Cardiol. 53(14):1204-10, 2009
                                                                              16. Puchalski MD et al: Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy? Int J Cardiovasc Imaging. 25(1):57-63, 2009
                                                                              17. Rhodes J et al: Safety and efficacy of carvedilol therapy for patients with dilated cardiomyopathy secondary to muscular dystrophy. Pediatr Cardiol. 29(2):343-51, 2008
                                                                              18. Silva MC et al: Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy. J Am Coll Cardiol. 49(18):1874-9, 2007
                                                                              19. Ashford MW Jr et al: Occult cardiac contractile dysfunction in dystrophin-deficient children revealed by cardiac magnetic resonance strain imaging. Circulation. 112(16):2462-7, 2005