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. 2013 May 21:7:97-114.
doi: 10.4137/CMC.S10940. Print 2013.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review

Jorge Romero  1 , Eliany Mejia-Lopez, Carlos Manrique, Richard Lucariello
Affiliations

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review

Jorge Romero et al. Clin Med Insights Cardiol. .

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for 11%-22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.

Keywords: Arrhythmogenic right ventricular cardiomyopathy (ARVC/D); cardiomyopathy; sudden cardiac death; tachyarrhythmias.

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Figures

Figure 1
Figure 1
Presenting symptoms and frequencies in patients with ARVC/D.
Figure 2
Figure 2
Incidences of ventricular involvement in ARVC/D. Classic form are mostly RV involvement, LV or Biventricular involvement.
Figure 3
Figure 3
Diagnosis of ARVC/D: major and minor criteria.
Figure 4
Figure 4
Epsilon wave: low amplitude positive deflection at the end of the QRS complex, usually seen in V1 and V2. If the epsilon wave is of large magnitude a reciprocal epsilon wave may be seen in V5 or V6, this may suggest that large part of the RV is depolarizing late.
Figure 5
Figure 5
Incidence of ECG findings in ARVC/D.
Figure 6
Figure 6
Diagnostic accuracy of noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging..
Figure 7
Figure 7
Delayed enhancement magnetic resonance imaging. 4-chamber and short axis views of DE-CMR showing delayed enhancement of the free right ventricular wall. Notes: The arrow indicates the area of delayed enhancement, which represents fibrofatty replacement. The figure on the right shows a significantly dilated RV.
Figure 8
Figure 8
The imaging is a Haematoxylin and eosin stained section and shows the typical fibrofatty infiltration of the ventricles in ARVC/D. Note: The fibroadipose replacement advances from the epicardium to the endocardium and is associated with myocardial atrophy and myocytes loss.
Figure 9
Figure 9
Brugada syndrome: characterized by a dynamic ST-segment elevation (accentuated J wave) in leads V1 to V3 of the ECG followed by negative T wave.
See this image and copyright information in PMC

References

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