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
Conte E et al: Left-dominant arrhythmogenic cardiomyopathy diagnosed at cardiac CT. J Cardiovasc Comput Tomogr. 14(5):e7-8, 2020
Gomes AC et al: Arrhythmogenic right ventricular cardiomyopathy: an exuberant case affecting both ventricles. Circ Cardiovasc Imaging. 13(9):e010243, 2020
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
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
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
Aliyari Ghasabeh M et al: Epicardial fat distribution assessed with cardiac CT in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Radiology. 289(3):641-8, 2018
Corrado D et al: Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 376(1):61-72, 2017
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
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
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
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
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
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
Marcus FI et al: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 121(13):1533-41, 2010
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KEY FACTS
Terminology
Imaging
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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
Conte E et al: Left-dominant arrhythmogenic cardiomyopathy diagnosed at cardiac CT. J Cardiovasc Comput Tomogr. 14(5):e7-8, 2020
Gomes AC et al: Arrhythmogenic right ventricular cardiomyopathy: an exuberant case affecting both ventricles. Circ Cardiovasc Imaging. 13(9):e010243, 2020
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
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
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
Aliyari Ghasabeh M et al: Epicardial fat distribution assessed with cardiac CT in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Radiology. 289(3):641-8, 2018
Corrado D et al: Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 376(1):61-72, 2017
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
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
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
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
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
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
Marcus FI et al: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 121(13):1533-41, 2010
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