Arrhythmogenic right ventricular dysplasia differential diagnosis: Difference between revisions

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==Overview==
 
== Overview ==
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
 
<br />
==Differential Diagnosis==
==Differential Diagnosis==
The differential diagnosis for the ventricular tachycardia due to ARVD include:
 
The differential diagnosis for the ventricular tachycardia due to ARVD include:<ref name="pmid28431055">{{cite journal| author=Novak J, Zorzi A, Castelletti S, Pantasis A, Rigato I, Corrado D | display-authors=etal| title=Electrocardiographic differentiation of idiopathic right ventricular outflow tract ectopy from early arrhythmogenic right ventricular cardiomyopathy. | journal=Europace | year= 2017 | volume= 19 | issue= 4 | pages= 622-628 | pmid=28431055 | doi=10.1093/europace/euw018 | pmc=5400079 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28431055  }}</ref><ref name="pmid26903598">{{cite journal| author=Saberniak J, Leren IS, Haland TF, Beitnes JO, Hopp E, Borgquist R | display-authors=etal| title=Comparison of patients with early-phase arrhythmogenic right ventricular cardiomyopathy and right ventricular outflow tract ventricular tachycardia. | journal=Eur Heart J Cardiovasc Imaging | year= 2017 | volume= 18 | issue= 1 | pages= 62-69 | pmid=26903598 | doi=10.1093/ehjci/jew014 | pmc=5217739 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26903598  }}</ref><ref name="pmid21835319">{{cite journal| author=Hoffmayer KS, Machado ON, Marcus GM, Yang Y, Johnson CJ, Ermakov S | display-authors=etal| title=Electrocardiographic comparison of ventricular arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy and right ventricular outflow tract tachycardia. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 8 | pages= 831-8 | pmid=21835319 | doi=10.1016/j.jacc.2011.05.017 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21835319  }}</ref><ref name="pmid24585727">{{cite journal| author=Philips B, Madhavan S, James CA, te Riele AS, Murray B, Tichnell C | display-authors=etal| title=Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. | journal=Circ Arrhythm Electrophysiol | year= 2014 | volume= 7 | issue= 2 | pages= 230-6 | pmid=24585727 | doi=10.1161/CIRCEP.113.000932 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24585727  }}</ref><ref name="pmid19232901">{{cite journal| author=Pieroni M, Dello Russo A, Marzo F, Pelargonio G, Casella M, Bellocci F | display-authors=etal| title=High prevalence of myocarditis mimicking arrhythmogenic right ventricular cardiomyopathy differential diagnosis by electroanatomic mapping-guided endomyocardial biopsy. | journal=J Am Coll Cardiol | year= 2009 | volume= 53 | issue= 8 | pages= 681-9 | pmid=19232901 | doi=10.1016/j.jacc.2008.11.017 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19232901  }}</ref>
 
* Congenital heart disease
* Congenital heart disease
** Repaired [[tetralogy of Fallot]]
** Repaired [[tetralogy of Fallot]]
** [[Ebstein's anomaly]]
** [[Ebstein's anomaly]]
** Uhl's anomaly
** [[Uhl anomaly]]
** [[Atrial septal defect]]
** [[Atrial septal defect]]
** Partial anomalous venous return
** [[Partial anomalous pulmonary venous return]]
* Acquired heart disease
* Acquired heart disease
** Tricuspid valve disease
** [[Tricuspid valve disease]]
** [[Pulmonary hypertension]]
** [[Pulmonary hypertension]]
** [[Right ventricular infarction]]
** [[Right ventricular infarction]]
** Bundle-branch re-entrant tachycardia
** [[Bundle branch reentrant ventricular tachycardia]]
* Miscellaneous
* Miscellaneous
** Pre-excited AV re-entry tachycardia
** Pre-excited AV re-entry tachycardia
** Idiopathic RVOT tachycardia
** Idiopathic RVOT ventricular tachycardia
 
 
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Disease}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Characteristics}}
! style="background: #4479BA; padding: 5px 5px;" colspan=1 | {{fontcolor|#FFFFFF|Signs and Symptoms}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Associated Conditions}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Histopathology}}
! style="background: #4479BA; padding: 5px 5px;" colspan=1 | {{fontcolor|#FFFFFF|Lab finding 
&
Other evaluation}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Prognosis}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | ARVC
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
|-
| style="padding: 5px 5px; background: #DCDCDC;" |RVOT
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
|-
| style="padding: 5px 5px; background: #DCDCDC;" |Sarcoidosis
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
| style="padding: 5px 5px; background: #F5F5F5;" |
*
|}
 
==Differentiating ARVD/C From RVOT VT==
Ventricular tachycardia associated with ARVD/C may be difficult to differentiate from RVOT VT. [[Electrocardiography|Electrocardiographic]] clues that support the diagnosis of ARVD/C include multifocal and/or polymorphic [[ventricular tachycardia|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 V<sub>1</sub> to V<sub>3</sub> 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]].


==References==
==References==
{{reflist|2}}
{{Reflist|2}}


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[[Category:Cardiology]]
[[Category:Electrophysiology]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date Cardiology]]

Latest revision as of 18:34, 20 May 2020

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


Overview

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

& Other evaluation

Prognosis
ARVC
RVOT
Sarcoidosis

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.

References

  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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