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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 97-114 |
| Number of pages | 18 |
| Journal | Clinical Medicine Insights: Cardiology |
| Volume | 7 |
| DOIs | |
| State | Published - May 21 2013 |
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Keywords
- Arrhythmogenic right ventricular cardiomyopathy (ARVC/D)
- Cardiomyopathy
- Sudden cardiac death
- Tachyarrhythmias
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
Cite this
Arrhythmogenic right ventricular cardiomyopathy (ARVC/D) : A systematic literature review. / Romero, Jorge E.; Mejia-Lopez, Eeliany; Manrique, Carlos R.; Lucariello, Richard J.
In: Clinical Medicine Insights: Cardiology, Vol. 7, 21.05.2013, p. 97-114.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Arrhythmogenic right ventricular cardiomyopathy (ARVC/D)
T2 - A systematic literature review
AU - Romero, Jorge E.
AU - Mejia-Lopez, Eeliany
AU - Manrique, Carlos R.
AU - Lucariello, Richard J.
PY - 2013/5/21
Y1 - 2013/5/21
N2 - 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.
AB - 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.
KW - Arrhythmogenic right ventricular cardiomyopathy (ARVC/D)
KW - Cardiomyopathy
KW - Sudden cardiac death
KW - Tachyarrhythmias
UR - http://www.scopus.com/inward/record.url?scp=84878195274&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84878195274&partnerID=8YFLogxK
U2 - 10.4137/CMC.S10940
DO - 10.4137/CMC.S10940
M3 - Article
VL - 7
SP - 97
EP - 114
JO - Clinical Medicine Insights: Cardiology
JF - Clinical Medicine Insights: Cardiology
SN - 1179-5468
ER -