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

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Abbreviations

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

          • Distinct entity caused by mutations in genes encoding desmosomal proteins (plakoglobin, plakophilin, desmophilin, and desmocollin)
            • Result is apoptosis and early cell death and replacement with fibrofatty tissue
            • Predominantly affects RV with variable LV involvement and 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. Corrado D et al: Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 376(1):61-72, 2017
                                        2. 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):237-253, 2017
                                        3. 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
                                        4. 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
                                        5. 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
                                        6. 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
                                        7. 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
                                        8. 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

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

                                                • Distinct entity caused by mutations in genes encoding desmosomal proteins (plakoglobin, plakophilin, desmophilin, and desmocollin)
                                                  • Result is apoptosis and early cell death and replacement with fibrofatty tissue
                                                  • Predominantly affects RV with variable LV involvement and 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. Corrado D et al: Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 376(1):61-72, 2017
                                                                              2. 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):237-253, 2017
                                                                              3. 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
                                                                              4. 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
                                                                              5. 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
                                                                              6. 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
                                                                              7. 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
                                                                              8. Marcus FI et al: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 121(13):1533-41, 2010