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Arrhythmogenic right ventricular cardiomyopathy (ARVC): cardiovascular magnetic resonance update.

te Riele AS, Tandri H, Bluemke DA - J Cardiovasc Magn Reson (2014)

Bottom Line: Pathogenic mutations in genes encoding the cardiac desmosome can be found in approximately 60% of index patients, leading to our current perception of ARVC as a desmosomal disease.Prevention of sudden cardiac death using implantable cardioverter-defibrillators is the most important management consideration.This purpose of this paper is to provide an updated review of our understanding of the genetics, diagnosis, current state-of-the-art CMR acquisition and analysis, and management of patients with ARVC.

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ABSTRACT
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is one of the most arrhythmogenic forms of inherited cardiomyopathy and a frequent cause of sudden death in the young. Affected individuals typically present between the second and fourth decade of life with arrhythmias coming from the right ventricle. Pathogenic mutations in genes encoding the cardiac desmosome can be found in approximately 60% of index patients, leading to our current perception of ARVC as a desmosomal disease. Although ARVC is known to preferentially affect the right ventricle, early and/or predominant left ventricular involvement is increasingly recognized. Diagnosis is made by combining multiple sources of diagnostic information as prescribed by the "Task Force" criteria. Recent research suggests that electrical abnormalities precede structural changes in ARVC. Cardiovascular Magnetic Resonance (CMR) is an ideal technique in ARVC workup, as it provides comprehensive information on cardiac morphology, function, and tissue characterization in a single investigation. Prevention of sudden cardiac death using implantable cardioverter-defibrillators is the most important management consideration. This purpose of this paper is to provide an updated review of our understanding of the genetics, diagnosis, current state-of-the-art CMR acquisition and analysis, and management of patients with ARVC.

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Horizontal long-axis (top panels) bright blood and late gadolinium enhancement images (bottom panels) in an ARVC subject with predominant left ventricular abnormalities. Note a dilated left ventricle in the bright blood images. Late enhancement is observed in a mid-myocardial pattern in the basal septum and basal lateral wall (arrows, bottom panels).
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Figure 3: Horizontal long-axis (top panels) bright blood and late gadolinium enhancement images (bottom panels) in an ARVC subject with predominant left ventricular abnormalities. Note a dilated left ventricle in the bright blood images. Late enhancement is observed in a mid-myocardial pattern in the basal septum and basal lateral wall (arrows, bottom panels).

Mentions: The advent of genetic testing and use of sensitive TFC have significantly enhanced our appreciation of the wide phenotypic spectrum of ARVC, and increased our awareness of non-classical (including left-dominant and biventricular) phenotypes. As a result, we now know that some ARVC subjects have early and predominant LV involvement (Figure 3) [19],[55]–[57]. LV involvement has even been reported in 76% of ARVC subjects, of whom the majority had advanced disease [58]. The disease is, therefore, increasingly being referred to as “Arrhythmogenic Cardiomyopathy”.


Arrhythmogenic right ventricular cardiomyopathy (ARVC): cardiovascular magnetic resonance update.

te Riele AS, Tandri H, Bluemke DA - J Cardiovasc Magn Reson (2014)

Horizontal long-axis (top panels) bright blood and late gadolinium enhancement images (bottom panels) in an ARVC subject with predominant left ventricular abnormalities. Note a dilated left ventricle in the bright blood images. Late enhancement is observed in a mid-myocardial pattern in the basal septum and basal lateral wall (arrows, bottom panels).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4222825&req=5

Figure 3: Horizontal long-axis (top panels) bright blood and late gadolinium enhancement images (bottom panels) in an ARVC subject with predominant left ventricular abnormalities. Note a dilated left ventricle in the bright blood images. Late enhancement is observed in a mid-myocardial pattern in the basal septum and basal lateral wall (arrows, bottom panels).
Mentions: The advent of genetic testing and use of sensitive TFC have significantly enhanced our appreciation of the wide phenotypic spectrum of ARVC, and increased our awareness of non-classical (including left-dominant and biventricular) phenotypes. As a result, we now know that some ARVC subjects have early and predominant LV involvement (Figure 3) [19],[55]–[57]. LV involvement has even been reported in 76% of ARVC subjects, of whom the majority had advanced disease [58]. The disease is, therefore, increasingly being referred to as “Arrhythmogenic Cardiomyopathy”.

Bottom Line: Pathogenic mutations in genes encoding the cardiac desmosome can be found in approximately 60% of index patients, leading to our current perception of ARVC as a desmosomal disease.Prevention of sudden cardiac death using implantable cardioverter-defibrillators is the most important management consideration.This purpose of this paper is to provide an updated review of our understanding of the genetics, diagnosis, current state-of-the-art CMR acquisition and analysis, and management of patients with ARVC.

View Article: PubMed Central - HTML - PubMed

ABSTRACT
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is one of the most arrhythmogenic forms of inherited cardiomyopathy and a frequent cause of sudden death in the young. Affected individuals typically present between the second and fourth decade of life with arrhythmias coming from the right ventricle. Pathogenic mutations in genes encoding the cardiac desmosome can be found in approximately 60% of index patients, leading to our current perception of ARVC as a desmosomal disease. Although ARVC is known to preferentially affect the right ventricle, early and/or predominant left ventricular involvement is increasingly recognized. Diagnosis is made by combining multiple sources of diagnostic information as prescribed by the "Task Force" criteria. Recent research suggests that electrical abnormalities precede structural changes in ARVC. Cardiovascular Magnetic Resonance (CMR) is an ideal technique in ARVC workup, as it provides comprehensive information on cardiac morphology, function, and tissue characterization in a single investigation. Prevention of sudden cardiac death using implantable cardioverter-defibrillators is the most important management consideration. This purpose of this paper is to provide an updated review of our understanding of the genetics, diagnosis, current state-of-the-art CMR acquisition and analysis, and management of patients with ARVC.

Show MeSH
Related in: MedlinePlus