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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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Four-chamber (top panels) and short-axis (bottom panels) bright blood images in an ARVC subject with predominant right ventricular abnormalities. End-diastolic images are shown in the left panels, end-systolic images in the right panels. Note subtricuspid dyskinesia in the end-systolic four-chamber image (arrow), and right ventricular free wall aneurysms (i.e. both systolic and diastolic bulging) in the short-axis image (arrows).
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Figure 1: Four-chamber (top panels) and short-axis (bottom panels) bright blood images in an ARVC subject with predominant right ventricular abnormalities. End-diastolic images are shown in the left panels, end-systolic images in the right panels. Note subtricuspid dyskinesia in the end-systolic four-chamber image (arrow), and right ventricular free wall aneurysms (i.e. both systolic and diastolic bulging) in the short-axis image (arrows).

Mentions: Most of our information about structural abnormalities in ARVC comes from studies in subjects with a predominant RV phenotype (Figure 1) [42]–[44]. Abnormalities in the RV in ARVC have been extensively described (reviewed in [15]). Besides global reduction in RV function and enlargement of the RV, more subtle regional disease of the RV has been variously described in the literature using a variety of terms (including focal bulges, microaneurysms, segmental dilatation, regional hypokinesis, etc.). In the current TFC, the terms “akinesia” (lack of motion) and “dyskinesia” (abnormal movement – instead of contracting in systole, that segment of myocardium bulges outward in systole) and “dyssynchronous” (regional peak contraction occurring at different times in adjacent myocardium) are used for all imaging modalities (CMR, echocardiography and angiography) to describe regional wall motion abnormalities in ARVC. Microaneurysms are not explicitly described in the revised TFC for CMR; overuse of this finding was considered by the Task Force members to be misinterpreted by CMR physicians resulting in false positive diagnoses. However, microaneurysms are characterized by regional akinesia or dyskinesia in the revised criteria.


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

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

Four-chamber (top panels) and short-axis (bottom panels) bright blood images in an ARVC subject with predominant right ventricular abnormalities. End-diastolic images are shown in the left panels, end-systolic images in the right panels. Note subtricuspid dyskinesia in the end-systolic four-chamber image (arrow), and right ventricular free wall aneurysms (i.e. both systolic and diastolic bulging) in the short-axis image (arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Four-chamber (top panels) and short-axis (bottom panels) bright blood images in an ARVC subject with predominant right ventricular abnormalities. End-diastolic images are shown in the left panels, end-systolic images in the right panels. Note subtricuspid dyskinesia in the end-systolic four-chamber image (arrow), and right ventricular free wall aneurysms (i.e. both systolic and diastolic bulging) in the short-axis image (arrows).
Mentions: Most of our information about structural abnormalities in ARVC comes from studies in subjects with a predominant RV phenotype (Figure 1) [42]–[44]. Abnormalities in the RV in ARVC have been extensively described (reviewed in [15]). Besides global reduction in RV function and enlargement of the RV, more subtle regional disease of the RV has been variously described in the literature using a variety of terms (including focal bulges, microaneurysms, segmental dilatation, regional hypokinesis, etc.). In the current TFC, the terms “akinesia” (lack of motion) and “dyskinesia” (abnormal movement – instead of contracting in systole, that segment of myocardium bulges outward in systole) and “dyssynchronous” (regional peak contraction occurring at different times in adjacent myocardium) are used for all imaging modalities (CMR, echocardiography and angiography) to describe regional wall motion abnormalities in ARVC. Microaneurysms are not explicitly described in the revised TFC for CMR; overuse of this finding was considered by the Task Force members to be misinterpreted by CMR physicians resulting in false positive diagnoses. However, microaneurysms are characterized by regional akinesia or dyskinesia in the revised criteria.

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