Arrhythmogenic right ventricular cardiomyopathy resident survival guide: Difference between revisions
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==Classification== | ==Classification== | ||
===Stages of disease=== | ===Stages of disease=== | ||
*Concealed phase: Subclinical asymptomatic phase | *<u>Concealed phase:</u> Subclinical asymptomatic phase | ||
*Overt electrical disorder: Palpitations, syncope and typically with symptomatic ventricular arrhythmias of RV | *<u>Overt electrical disorder:</u> Palpitations, syncope and typically with symptomatic ventricular arrhythmias of RV | ||
*RV failure: Progressive loss of RV myocardium due to fibro-fatty replacement impairs RV function, pump failure | *<u>RV failure:</u> Progressive loss of RV myocardium due to fibro-fatty replacement impairs RV function, pump failure | ||
*Biventricular failure: Involvement of the interventricular septum and LV causing congestive heart failure (HF) | *<u>Biventricular failure:</u> Involvement of the interventricular septum and LV causing congestive heart failure (HF)<ref name="pmid25548613">{{cite journal| author=Pinamonti B, Brun F, Mestroni L, Sinagra G| title=Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges. | journal=World J Cardiol | year= 2014 | volume= 6 | issue= 12 | pages= 1234-44 | pmid=25548613 | doi=10.4330/wjc.v6.i12.1234 | pmc=PMC4278158 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25548613 }} </ref> | ||
===Patterns of expression=== | |||
*<u>Classic ARVC:</u>Increased RV to LV volume ratio, more severe involvement of the RV, negative anterior T waves and ventricular arrhythmias with LBBB morphology | |||
*<u>Left-dominant arrhythmogenic cardiomyopathy (LDAC):</u>Predominantly involves the LV, LV wall motion abnormalities, chamber dilation, systolic impairment, and late gadolinium enhancement (LGE), ventricular arrhythmias of right bundle branch block (RBBB) morphology, (infero)-lateral T-wave inversion | |||
*<u>Biventricular arrhythmogenic:</u>Early and parallel involvement of the RV and LV, biventricular dilation and systolic impairment, ventricular arrhythmias of both RBBB and LBBB configuration may occur, ratio of RV to LV volume remains close to 1<ref name="pmid25548613">{{cite journal| author=Pinamonti B, Brun F, Mestroni L, Sinagra G| title=Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges. | journal=World J Cardiol | year= 2014 | volume= 6 | issue= 12 | pages= 1234-44 | pmid=25548613 | doi=10.4330/wjc.v6.i12.1234 | pmc=PMC4278158 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25548613 }} </ref> | |||
===Patterns of expression=== | |||
==Causes== | ==Causes== | ||
Revision as of 15:35, 10 March 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Steven Bellm, M.D. [2]
| Arrhythmogenic right ventricular cardiomyopathy resident survival guide Microchapters |
|---|
| Overview |
| Classification |
| Causes |
| FIRE |
| Diagnosis |
| Treatment |
| Do's |
| Dont's |
Overview
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement of the right ventricular myocardium. This puts patients into risk of life-threatening ventricular arrhythmias and slowly progressive ventricular dysfunction. The diagnostic is challenging. Diagnosis of ARVC relays on a scoring system, with major or minor criteria on the Revised Task Force Criteria. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are main goals of the treatment.[1]
Classification
Stages of disease
- Concealed phase: Subclinical asymptomatic phase
- Overt electrical disorder: Palpitations, syncope and typically with symptomatic ventricular arrhythmias of RV
- RV failure: Progressive loss of RV myocardium due to fibro-fatty replacement impairs RV function, pump failure
- Biventricular failure: Involvement of the interventricular septum and LV causing congestive heart failure (HF)[1]
Patterns of expression
- Classic ARVC:Increased RV to LV volume ratio, more severe involvement of the RV, negative anterior T waves and ventricular arrhythmias with LBBB morphology
- Left-dominant arrhythmogenic cardiomyopathy (LDAC):Predominantly involves the LV, LV wall motion abnormalities, chamber dilation, systolic impairment, and late gadolinium enhancement (LGE), ventricular arrhythmias of right bundle branch block (RBBB) morphology, (infero)-lateral T-wave inversion
- Biventricular arrhythmogenic:Early and parallel involvement of the RV and LV, biventricular dilation and systolic impairment, ventricular arrhythmias of both RBBB and LBBB configuration may occur, ratio of RV to LV volume remains close to 1[1]
Patterns of expression
Causes
Life Threatening Causes
Common Causes
FIRE: Focused Initial Rapid Evaluation
A Focused Initial Rapid Evaluation (FIRE) should be performed to identify patients in need of immediate intervention.
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Complete Diagnostic Approach
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Treatment
shown
hidden
Do's
Dont's
References
- ↑ 1.0 1.1 1.2 Pinamonti B, Brun F, Mestroni L, Sinagra G (2014). "Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges". World J Cardiol. 6 (12): 1234–44. doi:10.4330/wjc.v6.i12.1234. PMC 4278158. PMID 25548613.