familial arrhythmogenic right ventricular dysplasia; arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD, ARVC, ARVD/C, ARVC/D)
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Epidemiology
- accounts for a significant % of sudden cardiac death in young people, including athletes[2]
Pathology
- dilation of the right ventricle
- partial degeneration of the myocardium of the right ventricle
- replacement of ventricular myocardium with fatty & fibrous elements
- preferentially involves the right ventricular free wall
Genetics
- autosomal dominant
- associated with defects in TGFB3 (type 1)
- associated with defects in RYR2 (type 2)
Clinical manifestations
Diagnostic procedures
- electrocardiogram* - electrical instability
- T-wave inversion V1-V3
- frequent PVCs
- abnormal signal-averaged ECG*
- HOLTER*
- monomorphic ventricular tachycardia
- ventricular tachycarydia with left bundle branch morphology suggests arrhythmogenic origin is from the right ventricle
- monomorphic ventricular tachycardia
- echocardiogram*
- right ventricular systolic dysfunction
- abnormal right ventricular size & function
- right ventricular systolic dysfunction
- electrophysiology study
- right ventricular biopsy
- fibrofatty replacement of right ventricular myocardium
* these tests collectively with best yield[2]
Radiology
- angiography* (defining criteria)
- magnetic resonance imaging has been used
* one of the tests collectively with best yield[2]
Complications
Management
- beta-blocker
- other antiarrhymiv agents, sotalol, amiodarone
- syncope is an indication for an ICD
- abstinence from exercise
More general terms
More specific terms
- arrhythmogenic right ventricular cardiomyopathy type 10; familial arrhythmogenic right ventricular dysplasia type 10 (ARVC10, ARVD10)
- arrhythmogenic right ventricular cardiomyopathy type 11; familial arrhythmogenic right ventricular dysplasia type 11 (ARVC11, ARVD11)
- arrhythmogenic right ventricular cardiomyopathy type 12; familial arrhythmogenic right ventricular dysplasia type 12 (ARVC12, ARVD12)
- arrhythmogenic right ventricular cardiomyopathy type 9; familial arrhythmogenic right ventricular dysplasia type 9 (ARVC9, ARVD9)
- familial arrhythmogenic right ventricular dysplasia 8 (ARVD8)
References
- ↑ UniProt http://www.uniprot.org/uniprot/Q92736.html
- ↑ 2.0 2.1 2.2 2.3 Marcus FI et al Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: Results from the North American Multidisciplinary Study. Heart Rhythm 2009 Jul; 6:984. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19560088
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012
- ↑ Basso C, Corrado D, Marcus FI, Nava A, Thiene G. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009 Apr 11;373(9671):1289-300. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19362677