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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 bright blood image in an ARVC patient revealing left ventricular lateral wall fatty infiltration with myocardial wall thinning (arrowhead).
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Figure 4: Horizontal long-axis bright blood image in an ARVC patient revealing left ventricular lateral wall fatty infiltration with myocardial wall thinning (arrowhead).

Mentions: LV involvement in ARVC may manifest as late gadolinium enhancement (LGE), often involving the inferior and lateral walls without concomitant wall motion abnormalities [55],[57],[60]. Septal LGE is present in more than 50% of cases with left dominant ARVC, in contrast to the right dominant pattern in which septal involvement is unusual [55]. In addition, LV fatty infiltration was shown to be a prevalent finding in ARVC, often involving the subepicardial lateral LV and resulting in myocardial wall thinning (FigureĀ 4) [54],[61]. Early data by Dalal et al. already showed that LV fat in the lateral wall is very specific for ARVC mutation carriers [45]. Future studies are necessary to confirm these data, and further our understanding of LV abnormalities in ARVC.


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

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

Horizontal long-axis bright blood image in an ARVC patient revealing left ventricular lateral wall fatty infiltration with myocardial wall thinning (arrowhead).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: Horizontal long-axis bright blood image in an ARVC patient revealing left ventricular lateral wall fatty infiltration with myocardial wall thinning (arrowhead).
Mentions: LV involvement in ARVC may manifest as late gadolinium enhancement (LGE), often involving the inferior and lateral walls without concomitant wall motion abnormalities [55],[57],[60]. Septal LGE is present in more than 50% of cases with left dominant ARVC, in contrast to the right dominant pattern in which septal involvement is unusual [55]. In addition, LV fatty infiltration was shown to be a prevalent finding in ARVC, often involving the subepicardial lateral LV and resulting in myocardial wall thinning (FigureĀ 4) [54],[61]. Early data by Dalal et al. already showed that LV fat in the lateral wall is very specific for ARVC mutation carriers [45]. Future studies are necessary to confirm these data, and further our understanding of LV abnormalities in ARVC.

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