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Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Leila Rezai Gharai, MD; John D. Grizzard, MD; Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Abbreviations

          • Arrhythmogenic right ventricular cardiomyopathy (ARVC)
        • Synonyms

          • Formerly known as arrhythmogenic right ventricular dysplasia (ARVD)
          • Term arrhythmogenic cardiomyopathy (AC) has recently been proposed to include both left ventricular (LV) & right ventricular (RV) disease
        • Definitions

          • Distinct entity caused by mutations in genes encoding desmosomal proteins (plakoglobin, plakophilin, desmophilin, & desmocollin)
            • Result is apoptosis & early cell death with replacement by fibrofatty tissue
            • Predominantly affects RV with variable LV involvement; is associated with arrhythmias, including sudden cardiac death (SCD)

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Echocardiographic Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Conte E et al: Left-dominant arrhythmogenic cardiomyopathy diagnosed at cardiac CT. J Cardiovasc Comput Tomogr. 14(5):e7-8, 2020
                                        2. Gomes AC et al: Arrhythmogenic right ventricular cardiomyopathy: an exuberant case affecting both ventricles. Circ Cardiovasc Imaging. 13(9):e010243, 2020
                                        3. Khanji MY et al: Cardiovascular magnetic resonance imaging volume criteria for arrhythmogenic right ventricular cardiomyopathy: need for update? Eur Heart J. 41(14):1451, 2020
                                        4. Padrón-Barthe L et al: Severe cardiac dysfunction and death caused by arrhythmogenic right ventricular cardiomyopathy type 5 are improved by inhibition of glycogen synthase kinase-3β. Circulation. 140(14):1188-204, 2019
                                        5. Towbin JA et al: 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: executive summary. Heart Rhythm. 16(11):e373-407, 2019
                                        6. Aliyari Ghasabeh M et al: Epicardial fat distribution assessed with cardiac CT in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Radiology. 289(3):641-8, 2018
                                        7. Corrado D et al: Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 376(1):61-72, 2017
                                        8. Haugaa KH et al: Comprehensive multi-modality imaging approach in arrhythmogenic cardiomyopathy-an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 18(3):237-53, 2017
                                        9. Rastegar N et al: Fibrofatty changes: incidence at cardiac MR imaging in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Radiology. 280(2):405-12, 2016
                                        10. Te Riele ASJM et al: The value of cardiac magnetic resonance imaging in evaluation of pediatric patients for arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 66(7):873-4, 2015
                                        11. Philips B et al: Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. Circ Arrhythm Electrophysiol. 7(2):230-6, 2014
                                        12. Rastegar N et al: Cardiac MR findings and potential diagnostic pitfalls in patients evaluated for arrhythmogenic right ventricular cardiomyopathy. Radiographics. 34(6):1553-70, 2014
                                        13. Nakajima T et al: Utility of ECG-gated MDCT to differentiate patients with ARVC/D from patients with ventricular tachyarrhythmias. J Cardiovasc Comput Tomogr. 7(4):223-33, 2013
                                        14. Marcus FI et al: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 121(13):1533-41, 2010
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Arrhythmogenic right ventricular cardiomyopathy (ARVC)
                                              • Synonyms

                                                • Formerly known as arrhythmogenic right ventricular dysplasia (ARVD)
                                                • Term arrhythmogenic cardiomyopathy (AC) has recently been proposed to include both left ventricular (LV) & right ventricular (RV) disease
                                              • Definitions

                                                • Distinct entity caused by mutations in genes encoding desmosomal proteins (plakoglobin, plakophilin, desmophilin, & desmocollin)
                                                  • Result is apoptosis & early cell death with replacement by fibrofatty tissue
                                                  • Predominantly affects RV with variable LV involvement; is associated with arrhythmias, including sudden cardiac death (SCD)

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Echocardiographic Findings

                                                        • Angiographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Conte E et al: Left-dominant arrhythmogenic cardiomyopathy diagnosed at cardiac CT. J Cardiovasc Comput Tomogr. 14(5):e7-8, 2020
                                                                              2. Gomes AC et al: Arrhythmogenic right ventricular cardiomyopathy: an exuberant case affecting both ventricles. Circ Cardiovasc Imaging. 13(9):e010243, 2020
                                                                              3. Khanji MY et al: Cardiovascular magnetic resonance imaging volume criteria for arrhythmogenic right ventricular cardiomyopathy: need for update? Eur Heart J. 41(14):1451, 2020
                                                                              4. Padrón-Barthe L et al: Severe cardiac dysfunction and death caused by arrhythmogenic right ventricular cardiomyopathy type 5 are improved by inhibition of glycogen synthase kinase-3β. Circulation. 140(14):1188-204, 2019
                                                                              5. Towbin JA et al: 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: executive summary. Heart Rhythm. 16(11):e373-407, 2019
                                                                              6. Aliyari Ghasabeh M et al: Epicardial fat distribution assessed with cardiac CT in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Radiology. 289(3):641-8, 2018
                                                                              7. Corrado D et al: Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 376(1):61-72, 2017
                                                                              8. Haugaa KH et al: Comprehensive multi-modality imaging approach in arrhythmogenic cardiomyopathy-an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 18(3):237-53, 2017
                                                                              9. Rastegar N et al: Fibrofatty changes: incidence at cardiac MR imaging in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Radiology. 280(2):405-12, 2016
                                                                              10. Te Riele ASJM et al: The value of cardiac magnetic resonance imaging in evaluation of pediatric patients for arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 66(7):873-4, 2015
                                                                              11. Philips B et al: Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. Circ Arrhythm Electrophysiol. 7(2):230-6, 2014
                                                                              12. Rastegar N et al: Cardiac MR findings and potential diagnostic pitfalls in patients evaluated for arrhythmogenic right ventricular cardiomyopathy. Radiographics. 34(6):1553-70, 2014
                                                                              13. Nakajima T et al: Utility of ECG-gated MDCT to differentiate patients with ARVC/D from patients with ventricular tachyarrhythmias. J Cardiovasc Comput Tomogr. 7(4):223-33, 2013
                                                                              14. Marcus FI et al: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 121(13):1533-41, 2010