Arrhythmogenic right ventricular dysplasia differential diagnosis

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Differentiating Arrhythmogenic right ventricular dysplasia from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


The differential diagnosis of ARVC from other diseases can be challenging. It must be differentiated form RV outflow tract (RVOT) tachycardia or ectopy, Brugada syndrome, sarcoidosis, myocarditis, congenital abnormalities, pulmonary hypertension, RV infarct, DCM, Athlete heart, and Uhl's anomaly. At autopsy, one has to differentiate between normal fat deposition in the right ventricle and the presence of scars in the subepicardium due to ischemia

Differential Diagnosis

The differential diagnosis for the ventricular tachycardia due to ARVD include:[1][2][3][4][5]

Disease Characteristics Signs and Symptoms Associated Conditions Histopathology Lab finding

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Differentiating ARVD/C From RVOT VT

Ventricular tachycardia associated with ARVD/C may be difficult to differentiate from RVOT VT. Electrocardiographic clues that support the diagnosis of ARVD/C include multifocal and/or polymorphic ventricular tachycardia (VT) originating from the right ventricle, T wave inversions in the right precordial leads, slurred upstroke (≥55 ms) of the S wave in V1 to V3 leads, and the presence of an epsilon wave.

Differentiating ARVD/C From Dilated Cardiomyopathy

ARVD is distinguished from dilated cardiomyopathy by the greater degree of arrhythmogenicity. In dilated cardiomyopathy, although ventricular arrhythmia commonly occurs, it is rare in the absence of significant ventricular dysfunction. In contrast, ARVD is significantly associated with ventricular arrhythmia even in the absence of ventricular dysfunction. Furthermore, sudden cardiac death is the first clinical manifestation of the disease in more than 50% of probands with ARVD. Additionally, regional ventricular involvement and the presence of ventricular aneurysm, which are hallmarks of ARVD/C, argue against the diagnosis of dilated cardiomyopathy.


  1. Novak J, Zorzi A, Castelletti S, Pantasis A, Rigato I, Corrado D; et al. (2017). "Electrocardiographic differentiation of idiopathic right ventricular outflow tract ectopy from early arrhythmogenic right ventricular cardiomyopathy". Europace. 19 (4): 622–628. doi:10.1093/europace/euw018. PMC 5400079. PMID 28431055.
  2. Saberniak J, Leren IS, Haland TF, Beitnes JO, Hopp E, Borgquist R; et al. (2017). "Comparison of patients with early-phase arrhythmogenic right ventricular cardiomyopathy and right ventricular outflow tract ventricular tachycardia". Eur Heart J Cardiovasc Imaging. 18 (1): 62–69. doi:10.1093/ehjci/jew014. PMC 5217739. PMID 26903598.
  3. Hoffmayer KS, Machado ON, Marcus GM, Yang Y, Johnson CJ, Ermakov S; et al. (2011). "Electrocardiographic comparison of ventricular arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy and right ventricular outflow tract tachycardia". J Am Coll Cardiol. 58 (8): 831–8. doi:10.1016/j.jacc.2011.05.017. PMID 21835319.
  4. Philips B, Madhavan S, James CA, te Riele AS, Murray B, Tichnell C; et al. (2014). "Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear". Circ Arrhythm Electrophysiol. 7 (2): 230–6. doi:10.1161/CIRCEP.113.000932. PMID 24585727.
  5. Pieroni M, Dello Russo A, Marzo F, Pelargonio G, Casella M, Bellocci F; et al. (2009). "High prevalence of myocarditis mimicking arrhythmogenic right ventricular cardiomyopathy differential diagnosis by electroanatomic mapping-guided endomyocardial biopsy". J Am Coll Cardiol. 53 (8): 681–9. doi:10.1016/j.jacc.2008.11.017. PMID 19232901.

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