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 - Funding Information:
This work was performed during Dr. te Riele’s tenure as the Mark Josephson and Hein Wellens Research Fellow of the Heart Rhythm Society. Funding was received from the Dr. Francis P. Chiaramonte Private Foundation , St. Jude Medical, Inc. , and Medtronic, Inc . The Johns Hopkins ARVD/C Program is supported by the Leyla Erkan Family Fund for ARVD research, 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. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Publisher Copyright:
© 2015 American College of Cardiology Foundation.
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 -