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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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Regional contraction abnormality in the subtricuspid region. End diastolic (left) and end systolic image (right) show the so-called “accordion sign” in an ARVC mutation carrier. Regional dyssynchronous contraction in the subtricuspid region is a readily recognized qualitative pattern of abnormal RV contraction.
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Figure 2: Regional contraction abnormality in the subtricuspid region. End diastolic (left) and end systolic image (right) show the so-called “accordion sign” in an ARVC mutation carrier. Regional dyssynchronous contraction in the subtricuspid region is a readily recognized qualitative pattern of abnormal RV contraction.

Mentions: The location of regional wall motion abnormalities of the RV was not addressed in the revised TFC. We now recognize that the distal RV (from the moderator band to the apex in long axis views) shows highly variable contraction patterns in the normal individual. Therefore in ARVC, we emphasize the significance of regional wall motion abnormalities in the subtricuspid region. An excellent example of this is the so-called “accordion sign” that represents a focal “crinkling” of the myocardium (Figure 2) [45],[46]. In terms of TFC, the accordion sign is due to a small region of highly localized myocardium with dyssynchronous contraction.


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

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

Regional contraction abnormality in the subtricuspid region. End diastolic (left) and end systolic image (right) show the so-called “accordion sign” in an ARVC mutation carrier. Regional dyssynchronous contraction in the subtricuspid region is a readily recognized qualitative pattern of abnormal RV contraction.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Regional contraction abnormality in the subtricuspid region. End diastolic (left) and end systolic image (right) show the so-called “accordion sign” in an ARVC mutation carrier. Regional dyssynchronous contraction in the subtricuspid region is a readily recognized qualitative pattern of abnormal RV contraction.
Mentions: The location of regional wall motion abnormalities of the RV was not addressed in the revised TFC. We now recognize that the distal RV (from the moderator band to the apex in long axis views) shows highly variable contraction patterns in the normal individual. Therefore in ARVC, we emphasize the significance of regional wall motion abnormalities in the subtricuspid region. An excellent example of this is the so-called “accordion sign” that represents a focal “crinkling” of the myocardium (Figure 2) [45],[46]. In terms of TFC, the accordion sign is due to a small region of highly localized myocardium with dyssynchronous contraction.

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