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Restrictive Cardiomyopathy
Sanjeev A. Francis, MD, FACC; Suhny Abbara, MD, FACR, FSCCT
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Definitions

            • Abnormal diastolic function with relatively preserved systolic function
              • Normal ventricular cavity size
              • Abnormal ventricular compliance that leads to elevated filling pressures
              • Hemodynamics can resemble constrictive pericarditis

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Anderson HN et al: Idiopathic restrictive cardiomyopathy in children and young adults. Am J Cardiol. 121(10):1266-70, 2018
                                          2. Pereira NL et al: Spectrum of restrictive and infiltrative cardiomyopathies: part 2 of a 2-part series. J Am Coll Cardiol. 71(10):1149-66, 2018
                                          3. Pereira NL et al: Spectrum of restrictive and infiltrative cardiomyopathies: part 1 of a 2-part series. J Am Coll Cardiol. 71(10):1130-48, 2018
                                          4. Fulton N et al: Utility of magnetic resonance imaging in the evaluation of left ventricular thickening. Insights Imaging. 8(2):279-93, 2017
                                          5. Muchtar E et al: Restrictive cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy. Circ Res. 121(7):819-37, 2017
                                          6. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                          7. Gupta A et al: Cardiac MRI in restrictive cardiomyopathy. Clin Radiol. 67(2):95-105, 2012
                                          8. Quarta G et al: Cardiomyopathies: focus on cardiovascular magnetic resonance. Br J Radiol. 84 Spec No 3:S296-305, 2011
                                          9. Rochitte CE et al: The emerging role of MRI in the diagnosis and management of cardiomyopathies. Curr Cardiol Rep. 8(1):44-52, 2006
                                          10. Cury RC et al: Images in cardiovascular medicine. Visualization of endomyocardial fibrosis by delayed-enhancement magnetic resonance imaging. Circulation. 111(9):e115-7, 2005
                                          11. Chinnaiyan KM et al: Constrictive pericarditis versus restrictive cardiomyopathy: challenges in diagnosis and management. Cardiol Rev. 12(6):314-20, 2004
                                          12. Friedrich MG: Cardiovascular magnetic resonance in cardiomyopathies. In Manning WJ et al: Cardiovascular Magnetic Resonance. Philadelphia: Churchill Livingstone. 415-8, 2002
                                          13. Ammash NM et al: Clinical profile and outcome of idiopathic restrictive cardiomyopathy. Circulation. 101(21):2490-6, 2000
                                          14. Myers RB et al: Constrictive pericarditis: clinical and pathophysiologic characteristics. Am Heart J. 138(2 Pt 1):219-32, 1999
                                          15. Angelini A et al: Morphologic spectrum of primary restrictive cardiomyopathy. Am J Cardiol. 80(8):1046-50, 1997
                                          16. Kushwaha SS et al: Restrictive cardiomyopathy. N Engl J Med. 336(4):267-76, 1997
                                          17. Leung DY et al: Restrictive cardiomyopathy: diagnosis and prognostic implications. In Otto CM: The Practice of Clinical Echocardiography. Philadelphia: W. B. Saunders. 473-94, 1997
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Definitions

                                                    • Abnormal diastolic function with relatively preserved systolic function
                                                      • Normal ventricular cavity size
                                                      • Abnormal ventricular compliance that leads to elevated filling pressures
                                                      • Hemodynamics can resemble constrictive pericarditis

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Echocardiographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Anderson HN et al: Idiopathic restrictive cardiomyopathy in children and young adults. Am J Cardiol. 121(10):1266-70, 2018
                                                                                  2. Pereira NL et al: Spectrum of restrictive and infiltrative cardiomyopathies: part 2 of a 2-part series. J Am Coll Cardiol. 71(10):1149-66, 2018
                                                                                  3. Pereira NL et al: Spectrum of restrictive and infiltrative cardiomyopathies: part 1 of a 2-part series. J Am Coll Cardiol. 71(10):1130-48, 2018
                                                                                  4. Fulton N et al: Utility of magnetic resonance imaging in the evaluation of left ventricular thickening. Insights Imaging. 8(2):279-93, 2017
                                                                                  5. Muchtar E et al: Restrictive cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy. Circ Res. 121(7):819-37, 2017
                                                                                  6. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                                                                  7. Gupta A et al: Cardiac MRI in restrictive cardiomyopathy. Clin Radiol. 67(2):95-105, 2012
                                                                                  8. Quarta G et al: Cardiomyopathies: focus on cardiovascular magnetic resonance. Br J Radiol. 84 Spec No 3:S296-305, 2011
                                                                                  9. Rochitte CE et al: The emerging role of MRI in the diagnosis and management of cardiomyopathies. Curr Cardiol Rep. 8(1):44-52, 2006
                                                                                  10. Cury RC et al: Images in cardiovascular medicine. Visualization of endomyocardial fibrosis by delayed-enhancement magnetic resonance imaging. Circulation. 111(9):e115-7, 2005
                                                                                  11. Chinnaiyan KM et al: Constrictive pericarditis versus restrictive cardiomyopathy: challenges in diagnosis and management. Cardiol Rev. 12(6):314-20, 2004
                                                                                  12. Friedrich MG: Cardiovascular magnetic resonance in cardiomyopathies. In Manning WJ et al: Cardiovascular Magnetic Resonance. Philadelphia: Churchill Livingstone. 415-8, 2002
                                                                                  13. Ammash NM et al: Clinical profile and outcome of idiopathic restrictive cardiomyopathy. Circulation. 101(21):2490-6, 2000
                                                                                  14. Myers RB et al: Constrictive pericarditis: clinical and pathophysiologic characteristics. Am Heart J. 138(2 Pt 1):219-32, 1999
                                                                                  15. Angelini A et al: Morphologic spectrum of primary restrictive cardiomyopathy. Am J Cardiol. 80(8):1046-50, 1997
                                                                                  16. Kushwaha SS et al: Restrictive cardiomyopathy. N Engl J Med. 336(4):267-76, 1997
                                                                                  17. Leung DY et al: Restrictive cardiomyopathy: diagnosis and prognostic implications. In Otto CM: The Practice of Clinical Echocardiography. Philadelphia: W. B. Saunders. 473-94, 1997