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Hypertrophic Cardiomyopathy
Sanjeev A. Francis, MD, FACC; Suhny Abbara, MD, FACR, FSCCT; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Hypertrophic cardiomyopathy (HCM)
          • Synonyms

            • When left ventricular outflow tract (LVOT) obstruction is present, disease is referred to as
              • Hypertrophic obstructive cardiomyopathy (HOCM)
              • Idiopathic hypertrophic subaortic stenosis (IHSS)
          • Definitions

            • Genetic disease affecting proteins of myocardial sarcomere & associated myofilaments
              • Left ventricular (LV) hypertrophy without secondary cause
                • Hypertrophy can be present in various patterns, such as asymmetric septal (most common), concentric, midcavitary, or apical

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Nuclear Medicine 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

                                          Selected References

                                          1. Amano Y et al: Cardiac MR imaging of hypertrophic cardiomyopathy: techniques, findings, and clinical relevance. Magn Reson Med Sci. 17(2):120-31, 2018
                                          2. Maron BJ et al: Global burden of hypertrophic cardiomyopathy. JACC Heart Fail. 6(5):376-8, 2018
                                          3. Fulton N et al: Utility of magnetic resonance imaging in the evaluation of left ventricular thickening. Insights Imaging. 8(2):279-93, 2017
                                          4. Maron MS et al: How to image hypertrophic cardiomyopathy. Circ Cardiovasc Imaging. 10(7), 2017
                                          5. Baxi AJ et al: Hypertrophic cardiomyopathy from A to Z: genetics, pathophysiology, imaging, and management. Radiographics. 36(2):335-54, 2016
                                          6. Authors/Task Force members et al: 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 35(39):2733-79, 2014
                                          7. Chan RH et al: Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation. 130(6):484-95, 2014
                                          8. Noureldin RA et al: The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 14:17, 2012
                                          9. Gersh BJ et al: 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 124(24):e783-831, 2011
                                          10. Soler R et al: Magnetic resonance imaging of delayed enhancement in hypertrophic cardiomyopathy: relationship with left ventricular perfusion and contractile function. J Comput Assist Tomogr. 30(3):412-20, 2006
                                          11. Hughes SE: The pathology of hypertrophic cardiomyopathy. Histopathology. 44(5):412-27, 2004
                                          12. Moon JC et al: Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol. 41(9):1561-7, 2003
                                          13. Braunwald E et al: Contemporary evaluation and management of hypertrophic cardiomyopathy. Circulation. 106(11):1312-6, 2002
                                          14. Wilson JM et al: Magnetic resonance imaging of myocardial fibrosis in hypertrophic cardiomyopathy. Tex Heart Inst J. 29(3):176-80, 2002
                                          15. Casolo GC et al: Detection of apical hypertrophic cardiomyopathy by magnetic resonance imaging. Am Heart J. 117(2):468-72, 1989
                                          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

                                                    • Hypertrophic cardiomyopathy (HCM)
                                                  • Synonyms

                                                    • When left ventricular outflow tract (LVOT) obstruction is present, disease is referred to as
                                                      • Hypertrophic obstructive cardiomyopathy (HOCM)
                                                      • Idiopathic hypertrophic subaortic stenosis (IHSS)
                                                  • Definitions

                                                    • Genetic disease affecting proteins of myocardial sarcomere & associated myofilaments
                                                      • Left ventricular (LV) hypertrophy without secondary cause
                                                        • Hypertrophy can be present in various patterns, such as asymmetric septal (most common), concentric, midcavitary, or apical

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Echocardiographic Findings

                                                            • Nuclear Medicine 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

                                                                                  Selected References

                                                                                  1. Amano Y et al: Cardiac MR imaging of hypertrophic cardiomyopathy: techniques, findings, and clinical relevance. Magn Reson Med Sci. 17(2):120-31, 2018
                                                                                  2. Maron BJ et al: Global burden of hypertrophic cardiomyopathy. JACC Heart Fail. 6(5):376-8, 2018
                                                                                  3. Fulton N et al: Utility of magnetic resonance imaging in the evaluation of left ventricular thickening. Insights Imaging. 8(2):279-93, 2017
                                                                                  4. Maron MS et al: How to image hypertrophic cardiomyopathy. Circ Cardiovasc Imaging. 10(7), 2017
                                                                                  5. Baxi AJ et al: Hypertrophic cardiomyopathy from A to Z: genetics, pathophysiology, imaging, and management. Radiographics. 36(2):335-54, 2016
                                                                                  6. Authors/Task Force members et al: 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 35(39):2733-79, 2014
                                                                                  7. Chan RH et al: Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation. 130(6):484-95, 2014
                                                                                  8. Noureldin RA et al: The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 14:17, 2012
                                                                                  9. Gersh BJ et al: 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 124(24):e783-831, 2011
                                                                                  10. Soler R et al: Magnetic resonance imaging of delayed enhancement in hypertrophic cardiomyopathy: relationship with left ventricular perfusion and contractile function. J Comput Assist Tomogr. 30(3):412-20, 2006
                                                                                  11. Hughes SE: The pathology of hypertrophic cardiomyopathy. Histopathology. 44(5):412-27, 2004
                                                                                  12. Moon JC et al: Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol. 41(9):1561-7, 2003
                                                                                  13. Braunwald E et al: Contemporary evaluation and management of hypertrophic cardiomyopathy. Circulation. 106(11):1312-6, 2002
                                                                                  14. Wilson JM et al: Magnetic resonance imaging of myocardial fibrosis in hypertrophic cardiomyopathy. Tex Heart Inst J. 29(3):176-80, 2002
                                                                                  15. Casolo GC et al: Detection of apical hypertrophic cardiomyopathy by magnetic resonance imaging. Am Heart J. 117(2):468-72, 1989