TY - JOUR
T1 - Noninvasive multimodality imaging in ARVD/C
AU - Te Riele, Anneline S.J.M.
AU - Tandri, Harikrishna
AU - Sanborn, Danita M.
AU - Bluemke, David A.
N1 - Publisher Copyright:
© 2015 American College of Cardiology Foundation.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2015/5/1
Y1 - 2015/5/1
N2 - Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial cardiomyopathy resulting in progressive right ventricular (RV) dysfunction and malignant ventricular arrhythmias. Although ARVD/C is generally considered an inherited cardiomyopathy, the arrhythmogenic nature of the disease is striking. Affected individuals typically present in the second to fourth decade of life with arrhythmias originating from the right ventricle. Over the past decade, pathogenic ARVD/C-causing mutations have been identified in 5 genes encoding the cardiac desmosome. Disruption of the desmosomal connection system between cardiomyocytes may be represented structurally by ventricular enlargement, global or regional contraction abnormalities, RV aneurysms, or fibrofatty replacement. These abnormalities are typically observed in predilection areas, including the subtricuspid region, basal RV free wall, and left ventricular posterolateral wall. As such, structural and functional abnormalities on cardiac imaging constitute an important diagnostic criterion for the disease. This paper discusses the current status and role of echocardiography, cardiac magnetic resonance imaging, and computed tomography for suspected ARVD/C.
AB - Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial cardiomyopathy resulting in progressive right ventricular (RV) dysfunction and malignant ventricular arrhythmias. Although ARVD/C is generally considered an inherited cardiomyopathy, the arrhythmogenic nature of the disease is striking. Affected individuals typically present in the second to fourth decade of life with arrhythmias originating from the right ventricle. Over the past decade, pathogenic ARVD/C-causing mutations have been identified in 5 genes encoding the cardiac desmosome. Disruption of the desmosomal connection system between cardiomyocytes may be represented structurally by ventricular enlargement, global or regional contraction abnormalities, RV aneurysms, or fibrofatty replacement. These abnormalities are typically observed in predilection areas, including the subtricuspid region, basal RV free wall, and left ventricular posterolateral wall. As such, structural and functional abnormalities on cardiac imaging constitute an important diagnostic criterion for the disease. This paper discusses the current status and role of echocardiography, cardiac magnetic resonance imaging, and computed tomography for suspected ARVD/C.
KW - arrhythmogenic right ventricular dysplasia/cardiomyopathy
KW - cardiac magnetic resonance
KW - computed tomography
KW - echocardiography
KW - imaging
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U2 - 10.1016/j.jcmg.2015.02.007
DO - 10.1016/j.jcmg.2015.02.007
M3 - Review article
C2 - 25937197
AN - SCOPUS:84930006771
VL - 8
SP - 597
EP - 611
JO - JACC: Cardiovascular Imaging
JF - JACC: Cardiovascular Imaging
SN - 1936-878X
IS - 5
ER -