TY - JOUR
T1 - Arrhythmogenic right ventricular cardiomyopathy (ARVC)
T2 - Cardiovascular magnetic resonance update
AU - Te Riele, Anneline S.J.M.
AU - Tandri, Harikrishna
AU - Bluemke, David A.
N1 - Funding Information:
The authors wish to acknowledge funding from the Alexandre Suerman Stipend (to AT), the National Heart, Lung, and Blood Institute (K23HL093350 to HT), the Dr. Francis P. Chiaramonte Private Foundation, the St. Jude Medical Foundation, Medtronic Inc., and the NIH intramural research program (to DAB). The Johns Hopkins ARVC Program is supported by the Dr. Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins, the Bogle Foundation, the Healing Hearts Foundation, the Campanella family, the Patrick J. Harrison Family, the Peter French Memorial Foundation, and the Wilmerding Endowments. We are grateful to the ARVC patients and families who have made this work possible.
Publisher Copyright:
© 2014 te Riele et al.;licensee BioMed Central.
PY - 2014/7/20
Y1 - 2014/7/20
N2 - 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.
AB - 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.
KW - Arrhythmogenic right ventricular cardiomyopathy
KW - Cardiovascular magnetic resonance
KW - Diagnosis
KW - Genetics
KW - Treatment
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U2 - 10.1186/s12968-014-0050-8
DO - 10.1186/s12968-014-0050-8
M3 - Article
C2 - 25191878
AN - SCOPUS:84906559446
SN - 1097-6647
VL - 16
JO - Journal of Cardiovascular Magnetic Resonance
JF - Journal of Cardiovascular Magnetic Resonance
IS - 1
M1 - 50
ER -