Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced

Anneline S. J. M. Te Riele; Cynthia A. James; Binu Philips; Neda Rastegar; Aditya Bhonsale; Judith A. Groeneweg; Brittney Murray; Crystal Tichnell; Daniel P. Judge; Jeroen F.  van der Heijden; Maarten J. M. Cramer; Birgitta K. Velthuis; David A. Bluemke; Stefan L. Zimmerman; Ihab R. Kamel; Richard N. W. Hauer; Hugh Calkins; Harikrishna Tandri; JF van der Heijden

doi:10.1111/jce.12222

Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced

Anneline S. J. M. Te Riele, Cynthia A. James, Binu Philips, Neda Rastegar, Aditya Bhonsale, Judith A. Groeneweg, Brittney Murray, Crystal Tichnell, Daniel P. Judge, Jeroen F. van der Heijden, Maarten J. M. Cramer, Birgitta K. Velthuis, David A. Bluemke, Stefan L. Zimmerman, Ihab R. Kamel, Richard N. W. Hauer, Hugh Calkins, Harikrishna Tandri^*, JF van der Heijden

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

ARVD/C: The Triangle of Dysplasia Displaced

IntroductionThe traditional description of the Triangle of Dysplasia in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes.

Methods and ResultsWe analyzed Cardiac Magnetic Resonance (CMR) studies of 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV and 17-segment LV model. The location of electroanatomic endo- and epicardial scar and site of successful VT ablation was recorded in 11 ARVD/C subjects. Among 54/74 (73%) subjects with abnormal CMR, the RV was abnormal in almost all (96%), and 52% had biventricular involvement. Isolated LV abnormalities were uncommon (4%). Dyskinetic basal inferior wall (94%) was the most prevalent RV abnormality, followed by basal anterior wall (87%) dyskinesis. Subepicardial fat infiltration in the posterolateral LV (80%) was the most frequent LV abnormality. Similar to CMR data, voltage maps revealed scar (

ConclusionMutation-positive ARVD/C exhibits a previously unrecognized characteristic pattern of disease involving the basal inferior and anterior RV, and the posterolateral LV. The RV apex is only involved in advanced ARVD/C, typically as a part of global RV involvement. These results displace the RV apex from the Triangle of Dysplasia, and provide insights into the pathophysiology of ARVD/C.

Original language	English
Pages (from-to)	1311-1320
Number of pages	10
Journal	Journal of Cardiovascular Electrophysiology
Volume	24
Issue number	12
DOIs	https://doi.org/10.1111/jce.12222
Publication status	Published - Dec 2013

Keywords

arrhythmogenic right ventricular dysplasia
cardiomyopathy
magnetic resonance imaging
electroanatomic mapping
ventricular tachcardia
phenotype
genetics
implantable cardioverter defibrillator
TASK-FORCE CRITERIA
MAGNETIC-RESONANCE
HUMAN HEART
CARDIOMYOPATHY
TACHYCARDIA
ABLATION
SUBSTRATE
FAMILIES
DISEASE

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10.1111/jce.12222

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Te Riele, A. S. J. M., James, C. A., Philips, B., Rastegar, N., Bhonsale, A., Groeneweg, J. A., Murray, B., Tichnell, C., Judge, D. P., van der Heijden, J. F., Cramer, M. J. M., Velthuis, B. K., Bluemke, D. A., Zimmerman, S. L., Kamel, I. R., Hauer, R. N. W., Calkins, H., Tandri, H., & van der Heijden, JF. (2013). Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced. Journal of Cardiovascular Electrophysiology, 24(12), 1311-1320. https://doi.org/10.1111/jce.12222

@article{ea3011c3b14945a2a81dd796cb06079e,

title = "Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced",

abstract = "ARVD/C: The Triangle of Dysplasia DisplacedIntroductionThe traditional description of the Triangle of Dysplasia in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes.Methods and ResultsWe analyzed Cardiac Magnetic Resonance (CMR) studies of 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV and 17-segment LV model. The location of electroanatomic endo- and epicardial scar and site of successful VT ablation was recorded in 11 ARVD/C subjects. Among 54/74 (73%) subjects with abnormal CMR, the RV was abnormal in almost all (96%), and 52% had biventricular involvement. Isolated LV abnormalities were uncommon (4%). Dyskinetic basal inferior wall (94%) was the most prevalent RV abnormality, followed by basal anterior wall (87%) dyskinesis. Subepicardial fat infiltration in the posterolateral LV (80%) was the most frequent LV abnormality. Similar to CMR data, voltage maps revealed scar (ConclusionMutation-positive ARVD/C exhibits a previously unrecognized characteristic pattern of disease involving the basal inferior and anterior RV, and the posterolateral LV. The RV apex is only involved in advanced ARVD/C, typically as a part of global RV involvement. These results displace the RV apex from the Triangle of Dysplasia, and provide insights into the pathophysiology of ARVD/C.",

keywords = "arrhythmogenic right ventricular dysplasia, cardiomyopathy, magnetic resonance imaging, electroanatomic mapping, ventricular tachcardia, phenotype, genetics, implantable cardioverter defibrillator, TASK-FORCE CRITERIA, MAGNETIC-RESONANCE, HUMAN HEART, CARDIOMYOPATHY, TACHYCARDIA, ABLATION, SUBSTRATE, FAMILIES, DISEASE",

author = "{Te Riele}, {Anneline S. J. M.} and James, {Cynthia A.} and Binu Philips and Neda Rastegar and Aditya Bhonsale and Groeneweg, {Judith A.} and Brittney Murray and Crystal Tichnell and Judge, {Daniel P.} and {van der Heijden}, {Jeroen F.} and Cramer, {Maarten J. M.} and Velthuis, {Birgitta K.} and Bluemke, {David A.} and Zimmerman, {Stefan L.} and Kamel, {Ihab R.} and Hauer, {Richard N. W.} and Hugh Calkins and Harikrishna Tandri and {van der Heijden}, JF",

year = "2013",

month = dec,

doi = "10.1111/jce.12222",

language = "English",

volume = "24",

pages = "1311--1320",

journal = "Journal of Cardiovascular Electrophysiology",

issn = "1045-3873",

publisher = "Wiley-Blackwell",

number = "12",

}

Te Riele, ASJM, James, CA, Philips, B, Rastegar, N, Bhonsale, A, Groeneweg, JA, Murray, B, Tichnell, C, Judge, DP, van der Heijden, JF, Cramer, MJM, Velthuis, BK, Bluemke, DA, Zimmerman, SL, Kamel, IR, Hauer, RNW, Calkins, H, Tandri, H & van der Heijden, JF 2013, 'Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced', Journal of Cardiovascular Electrophysiology, vol. 24, no. 12, pp. 1311-1320. https://doi.org/10.1111/jce.12222

TY - JOUR

T1 - Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

T2 - The Triangle of Dysplasia Displaced

AU - Te Riele, Anneline S. J. M.

AU - James, Cynthia A.

AU - Philips, Binu

AU - Rastegar, Neda

AU - Bhonsale, Aditya

AU - Groeneweg, Judith A.

AU - Murray, Brittney

AU - Tichnell, Crystal

AU - Judge, Daniel P.

AU - van der Heijden, Jeroen F.

AU - Cramer, Maarten J. M.

AU - Velthuis, Birgitta K.

AU - Bluemke, David A.

AU - Zimmerman, Stefan L.

AU - Kamel, Ihab R.

AU - Hauer, Richard N. W.

AU - Calkins, Hugh

AU - Tandri, Harikrishna

AU - van der Heijden, JF

PY - 2013/12

Y1 - 2013/12

N2 - ARVD/C: The Triangle of Dysplasia DisplacedIntroductionThe traditional description of the Triangle of Dysplasia in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes.Methods and ResultsWe analyzed Cardiac Magnetic Resonance (CMR) studies of 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV and 17-segment LV model. The location of electroanatomic endo- and epicardial scar and site of successful VT ablation was recorded in 11 ARVD/C subjects. Among 54/74 (73%) subjects with abnormal CMR, the RV was abnormal in almost all (96%), and 52% had biventricular involvement. Isolated LV abnormalities were uncommon (4%). Dyskinetic basal inferior wall (94%) was the most prevalent RV abnormality, followed by basal anterior wall (87%) dyskinesis. Subepicardial fat infiltration in the posterolateral LV (80%) was the most frequent LV abnormality. Similar to CMR data, voltage maps revealed scar (ConclusionMutation-positive ARVD/C exhibits a previously unrecognized characteristic pattern of disease involving the basal inferior and anterior RV, and the posterolateral LV. The RV apex is only involved in advanced ARVD/C, typically as a part of global RV involvement. These results displace the RV apex from the Triangle of Dysplasia, and provide insights into the pathophysiology of ARVD/C.

AB - ARVD/C: The Triangle of Dysplasia DisplacedIntroductionThe traditional description of the Triangle of Dysplasia in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes.Methods and ResultsWe analyzed Cardiac Magnetic Resonance (CMR) studies of 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV and 17-segment LV model. The location of electroanatomic endo- and epicardial scar and site of successful VT ablation was recorded in 11 ARVD/C subjects. Among 54/74 (73%) subjects with abnormal CMR, the RV was abnormal in almost all (96%), and 52% had biventricular involvement. Isolated LV abnormalities were uncommon (4%). Dyskinetic basal inferior wall (94%) was the most prevalent RV abnormality, followed by basal anterior wall (87%) dyskinesis. Subepicardial fat infiltration in the posterolateral LV (80%) was the most frequent LV abnormality. Similar to CMR data, voltage maps revealed scar (ConclusionMutation-positive ARVD/C exhibits a previously unrecognized characteristic pattern of disease involving the basal inferior and anterior RV, and the posterolateral LV. The RV apex is only involved in advanced ARVD/C, typically as a part of global RV involvement. These results displace the RV apex from the Triangle of Dysplasia, and provide insights into the pathophysiology of ARVD/C.

KW - arrhythmogenic right ventricular dysplasia

KW - cardiomyopathy

KW - magnetic resonance imaging

KW - electroanatomic mapping

KW - ventricular tachcardia

KW - phenotype

KW - genetics

KW - implantable cardioverter defibrillator

KW - TASK-FORCE CRITERIA

KW - MAGNETIC-RESONANCE

KW - HUMAN HEART

KW - CARDIOMYOPATHY

KW - TACHYCARDIA

KW - ABLATION

KW - SUBSTRATE

KW - FAMILIES

KW - DISEASE

U2 - 10.1111/jce.12222

DO - 10.1111/jce.12222

M3 - Article

C2 - 23889974

SN - 1045-3873

VL - 24

SP - 1311

EP - 1320

JO - Journal of Cardiovascular Electrophysiology

JF - Journal of Cardiovascular Electrophysiology

IS - 12

ER -

Mutation-Positive Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: The Triangle of Dysplasia Displaced

Abstract

Keywords

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