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 = "John Wiley \& Sons Inc.",

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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