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

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Dilated cardiomyopathy (DCM)
        • Synonyms

          • Idiopathic dilated cardiomyopathy
        • Definitions

          • Left ventricular (LV) chamber dilation with systolic dysfunction ± right ventricular (RV) dilation/dysfunction
            • LV dilatation is assessed by LV diameter (2D echocardiography) or quantitative LV volumes [cardiac MR (CMR), 3D echo, MUGA]
            • Systolic dysfunction [LV ejection fraction (EF) < 55%]
          • Multiple possible etiologies
            • Idiopathic
            • Familial (genetic)
            • Infectious
            • Toxic (e.g., alcohol, cocaine)
            • Metabolic (e.g., hypophosphatemia, hypocalcemia, uremia)
            • Myocarditis
            • Infiltrative disease (e.g., hemochromatosis, sarcoid)
            • Peripartum cardiomyopathy
            • Connective tissue disease
            • Chemotherapy (e.g., doxorubicin)
            • Endocrinopathies (e.g., thyroid dysfunction, pheochromocytoma, Cushing disease)
            • Current definition excludes ischemic, hypertensive, and valvular heart disease

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Echocardiographic Findings

                  • Angiographic 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

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Brown PF et al: Towards cardiac MRI based risk stratification in idiopathic dilated cardiomyopathy. Heart. ePub, 2018
                                              2. Halliday BP et al: Personalizing Risk Stratification for Sudden Death in Dilated Cardiomyopathy: The Past, Present, and Future. Circulation. 136(2):215-231, 2017
                                              3. Youn JC et al: Contrast-enhanced T1 mapping-based extracellular volume fraction independently predicts clinical outcome in patients with non-ischemic dilated cardiomyopathy: a prospective cohort study. Eur Radiol. 27(9):3924-3933, 2017
                                              4. Disertori M et al: Myocardial fibrosis assessment by LGE is a powerful predictor of ventricular tachyarrhythmias in ischemic and nonischemic LV dysfunction: a meta-analysis. JACC Cardiovasc Imaging. 9(9):1046-55, 2016
                                              5. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                              6. Gulati A et al: Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy. JAMA. 309(9):896-908, 2013
                                              7. Puntmann VO et al: Native T1 mapping in differentiation of normal myocardium from diffuse disease in hypertrophic and dilated cardiomyopathy. JACC Cardiovasc Imaging. 6(4):475-84, 2013
                                              8. Ismail TF et al: Prognostic importance of late gadolinium enhancement cardiovascular magnetic resonance in cardiomyopathy. Heart. 98(6):438-42, 2012
                                              9. Colletti PM: Cardiac imaging 2006. AJR Am J Roentgenol. 186(6 Suppl 2):S337-9, 2006
                                              10. Gottlieb I et al: Magnetic resonance imaging in the evaluation of non-ischemic cardiomyopathies: current applications and future perspectives. Heart Fail Rev. 11(4):313-23, 2006
                                              11. Rochitte CE et al: The emerging role of MRI in the diagnosis and management of cardiomyopathies. Curr Cardiol Rep. 8(1):44-52, 2006
                                              12. Abbara S et al: Pericardial and myocardial disease. In Miller SW: Cardiac Imaging: The Requisites. 2nd ed. Philadelphia: Mosby. 270-2, 2005
                                              13. Zipes DP et al: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 7th ed. Philadelphia: Saunders/Elsevier, 2005
                                              14. McCrohon JA et al: Differentiation of heart failure related to dilated cardiomyopathy and coronary artery disease using gadolinium-enhanced cardiovascular magnetic resonance. Circulation. 108(1):54-9, 2003
                                              15. Felker GM et al: Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med. 342(15):1077-84, 2000
                                              16. Cooke CE: Idiopathic dilated cardiomyopathy. N Engl J Med. 332(20):1384; author reply 1385-6, 1995
                                              17. Dec GW et al: Idiopathic dilated cardiomyopathy. N Engl J Med. 331(23):1564-75, 1994
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Dilated cardiomyopathy (DCM)
                                                    • Synonyms

                                                      • Idiopathic dilated cardiomyopathy
                                                    • Definitions

                                                      • Left ventricular (LV) chamber dilation with systolic dysfunction ± right ventricular (RV) dilation/dysfunction
                                                        • LV dilatation is assessed by LV diameter (2D echocardiography) or quantitative LV volumes [cardiac MR (CMR), 3D echo, MUGA]
                                                        • Systolic dysfunction [LV ejection fraction (EF) < 55%]
                                                      • Multiple possible etiologies
                                                        • Idiopathic
                                                        • Familial (genetic)
                                                        • Infectious
                                                        • Toxic (e.g., alcohol, cocaine)
                                                        • Metabolic (e.g., hypophosphatemia, hypocalcemia, uremia)
                                                        • Myocarditis
                                                        • Infiltrative disease (e.g., hemochromatosis, sarcoid)
                                                        • Peripartum cardiomyopathy
                                                        • Connective tissue disease
                                                        • Chemotherapy (e.g., doxorubicin)
                                                        • Endocrinopathies (e.g., thyroid dysfunction, pheochromocytoma, Cushing disease)
                                                        • Current definition excludes ischemic, hypertensive, and valvular heart disease

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Echocardiographic Findings

                                                              • Angiographic 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

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Brown PF et al: Towards cardiac MRI based risk stratification in idiopathic dilated cardiomyopathy. Heart. ePub, 2018
                                                                                          2. Halliday BP et al: Personalizing Risk Stratification for Sudden Death in Dilated Cardiomyopathy: The Past, Present, and Future. Circulation. 136(2):215-231, 2017
                                                                                          3. Youn JC et al: Contrast-enhanced T1 mapping-based extracellular volume fraction independently predicts clinical outcome in patients with non-ischemic dilated cardiomyopathy: a prospective cohort study. Eur Radiol. 27(9):3924-3933, 2017
                                                                                          4. Disertori M et al: Myocardial fibrosis assessment by LGE is a powerful predictor of ventricular tachyarrhythmias in ischemic and nonischemic LV dysfunction: a meta-analysis. JACC Cardiovasc Imaging. 9(9):1046-55, 2016
                                                                                          5. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                                                                          6. Gulati A et al: Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy. JAMA. 309(9):896-908, 2013
                                                                                          7. Puntmann VO et al: Native T1 mapping in differentiation of normal myocardium from diffuse disease in hypertrophic and dilated cardiomyopathy. JACC Cardiovasc Imaging. 6(4):475-84, 2013
                                                                                          8. Ismail TF et al: Prognostic importance of late gadolinium enhancement cardiovascular magnetic resonance in cardiomyopathy. Heart. 98(6):438-42, 2012
                                                                                          9. Colletti PM: Cardiac imaging 2006. AJR Am J Roentgenol. 186(6 Suppl 2):S337-9, 2006
                                                                                          10. Gottlieb I et al: Magnetic resonance imaging in the evaluation of non-ischemic cardiomyopathies: current applications and future perspectives. Heart Fail Rev. 11(4):313-23, 2006
                                                                                          11. Rochitte CE et al: The emerging role of MRI in the diagnosis and management of cardiomyopathies. Curr Cardiol Rep. 8(1):44-52, 2006
                                                                                          12. Abbara S et al: Pericardial and myocardial disease. In Miller SW: Cardiac Imaging: The Requisites. 2nd ed. Philadelphia: Mosby. 270-2, 2005
                                                                                          13. Zipes DP et al: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 7th ed. Philadelphia: Saunders/Elsevier, 2005
                                                                                          14. McCrohon JA et al: Differentiation of heart failure related to dilated cardiomyopathy and coronary artery disease using gadolinium-enhanced cardiovascular magnetic resonance. Circulation. 108(1):54-9, 2003
                                                                                          15. Felker GM et al: Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med. 342(15):1077-84, 2000
                                                                                          16. Cooke CE: Idiopathic dilated cardiomyopathy. N Engl J Med. 332(20):1384; author reply 1385-6, 1995
                                                                                          17. Dec GW et al: Idiopathic dilated cardiomyopathy. N Engl J Med. 331(23):1564-75, 1994