The Prognostic Value of Right Ventricular Deformation Imaging in Early Arrhythmogenic Right Ventricular Cardiomyopathy

Thomas P. Mast; Karim Taha; Maarten J. Cramer; Joost Lumens; Jeroen F. van der Heijden; Berto J. Bouma; Maarten P. van den Berg; Folkert W. Asselbergs; Pieter A. Doevendans; Arco J. Teske

doi:10.1016/j.jcmg.2018.01.012

The Prognostic Value of Right Ventricular Deformation Imaging in Early Arrhythmogenic Right Ventricular Cardiomyopathy

Thomas P. Mast, Karim Taha, Maarten J. Cramer, Joost Lumens, Jeroen F. van der Heijden, Berto J. Bouma, Maarten P. van den Berg, Folkert W. Asselbergs, Pieter A. Doevendans, Arco J. Teske^*

^*Corresponding author for this work

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

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Abstract

OBJECTIVES The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols.

BACKGROUND ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC.

METHODS First-degree relatives of patients with ARVC were evaluated according to 2010 task force criteria, including RV deformation imaging (n = 128). Relatives fulfilling structural task force criteria were excluded for further analysis. At baseline, deformation patterns of the subtricuspid region were scored as type I (normal deformation), type II (delayed onset, decreased systolic peak, and post-systolic shortening), or type III (systolic stretching and large post-systolic shortening). The final study population comprised relatives who underwent a second evaluation during follow-up. Disease progression was defined as the development of a new 2010 task force criterion during follow-up that was absent at baseline.

RESULTS Sixty-five relatives underwent a second evaluation after a mean follow-up period of 3.7 +/- 2.1 years. At baseline, 28 relatives (43%) had normal deformation (type I), and 37 relatives (57%) had abnormal deformation (type II or III) in the subtricuspid region. Disease progression occurred in 4% of the relatives with normal deformation at baseline and in 43% of the relatives with abnormal deformation at baseline (p <0.001). Positive and negative predictive values of abnormal deformation were, respectively, 43% (95% confidence interval: 27% to 61%) and 96% (95% confidence interval: 82% to 100%).

CONCLUSIONS Normal RV deformation in the subtricuspid region is associated with absence of disease progression during nearly 4-year follow-up in relatives of patients with ARVC. Abnormal RV deformation seems to precede the established signs of ARVC. RV deformation imaging may potentially play an important role in ARVC family screening protocols. (C) 2019 by the American College of Cardiology Foundation.

Original language	English
Pages (from-to)	446-455
Number of pages	10
Journal	JACC-Cardiovascular Imaging
Volume	12
Issue number	3
DOIs	https://doi.org/10.1016/j.jcmg.2018.01.012
Publication status	Published - Mar 2019

Keywords

arrhythmogenic right ventricular cardiomyopathy
ARVD/C
deformation imaging
disease progression
family screening
strain imaging
TASK-FORCE CRITERIA
RISK STRATIFICATION
DYSPLASIA/CARDIOMYOPATHY
ECHOCARDIOGRAPHY

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10.1016/j.jcmg.2018.01.012

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Mast, T. P., Taha, K., Cramer, M. J., Lumens, J., van der Heijden, J. F., Bouma, B. J., van den Berg, M. P., Asselbergs, F. W., Doevendans, P. A., & Teske, A. J. (2019). The Prognostic Value of Right Ventricular Deformation Imaging in Early Arrhythmogenic Right Ventricular Cardiomyopathy. JACC-Cardiovascular Imaging, 12(3), 446-455. https://doi.org/10.1016/j.jcmg.2018.01.012

@article{db6c0cb819034c969bf81adbc7fa2f30,

title = "The Prognostic Value of Right Ventricular Deformation Imaging in Early Arrhythmogenic Right Ventricular Cardiomyopathy",

abstract = "OBJECTIVES The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols.BACKGROUND ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC.METHODS First-degree relatives of patients with ARVC were evaluated according to 2010 task force criteria, including RV deformation imaging (n = 128). Relatives fulfilling structural task force criteria were excluded for further analysis. At baseline, deformation patterns of the subtricuspid region were scored as type I (normal deformation), type II (delayed onset, decreased systolic peak, and post-systolic shortening), or type III (systolic stretching and large post-systolic shortening). The final study population comprised relatives who underwent a second evaluation during follow-up. Disease progression was defined as the development of a new 2010 task force criterion during follow-up that was absent at baseline.RESULTS Sixty-five relatives underwent a second evaluation after a mean follow-up period of 3.7 +/- 2.1 years. At baseline, 28 relatives (43%) had normal deformation (type I), and 37 relatives (57%) had abnormal deformation (type II or III) in the subtricuspid region. Disease progression occurred in 4% of the relatives with normal deformation at baseline and in 43% of the relatives with abnormal deformation at baseline (p <0.001). Positive and negative predictive values of abnormal deformation were, respectively, 43% (95% confidence interval: 27% to 61%) and 96% (95% confidence interval: 82% to 100%).CONCLUSIONS Normal RV deformation in the subtricuspid region is associated with absence of disease progression during nearly 4-year follow-up in relatives of patients with ARVC. Abnormal RV deformation seems to precede the established signs of ARVC. RV deformation imaging may potentially play an important role in ARVC family screening protocols. (C) 2019 by the American College of Cardiology Foundation.",

keywords = "arrhythmogenic right ventricular cardiomyopathy, ARVD/C, deformation imaging, disease progression, family screening, strain imaging, TASK-FORCE CRITERIA, RISK STRATIFICATION, DYSPLASIA/CARDIOMYOPATHY, ECHOCARDIOGRAPHY",

author = "Mast, {Thomas P.} and Karim Taha and Cramer, {Maarten J.} and Joost Lumens and {van der Heijden}, {Jeroen F.} and Bouma, {Berto J.} and {van den Berg}, {Maarten P.} and Asselbergs, {Folkert W.} and Doevendans, {Pieter A.} and Teske, {Arco J.}",

note = "Funding Information: This work was supported by the Netherlands Cardiovascular Research Initiative, an initiative with support from the Dutch Heart Foundation (CVON2015-12 eDETECT). Dr. Asselbergs is supported by a Dekker scholarship (Junior Staff Member 2014T001, Dutch Heart Foundation) and UCL Hospitals NIHR Biomedical Research Centre. Dr. Lumens has received a grant within the framework of the Dr. E. Dekker program of the Dutch Heart Foundation (NHS-2015T082). Funding Information: This work was supported by the Netherlands Cardiovascular Research Initiative, an initiative with support from the Dutch Heart Foundation (CVON2015-12 eDETECT). Dr. Asselbergs is supported by a Dekker scholarship (Junior Staff Member 2014T001, Dutch Heart Foundation) and UCL Hospitals NIHR Biomedical Research Centre. Dr. Lumens has received a grant within the framework of the Dr. E. Dekker program of the Dutch Heart Foundation (NHS-2015T082). Dr. Mast and Mr. Taha contributed equally to this work and are joint first authors. Publisher Copyright: {\textcopyright} 2019 American College of Cardiology Foundation",

year = "2019",

month = mar,

doi = "10.1016/j.jcmg.2018.01.012",

language = "English",

volume = "12",

pages = "446--455",

journal = "JACC-Cardiovascular Imaging",

issn = "1936-878X",

publisher = "Elsevier Science",

number = "3",

}

TY - JOUR

T1 - The Prognostic Value of Right Ventricular Deformation Imaging in Early Arrhythmogenic Right Ventricular Cardiomyopathy

AU - Mast, Thomas P.

AU - Taha, Karim

AU - Cramer, Maarten J.

AU - Lumens, Joost

AU - van der Heijden, Jeroen F.

AU - Bouma, Berto J.

AU - van den Berg, Maarten P.

AU - Asselbergs, Folkert W.

AU - Doevendans, Pieter A.

AU - Teske, Arco J.

N1 - Funding Information: This work was supported by the Netherlands Cardiovascular Research Initiative, an initiative with support from the Dutch Heart Foundation (CVON2015-12 eDETECT). Dr. Asselbergs is supported by a Dekker scholarship (Junior Staff Member 2014T001, Dutch Heart Foundation) and UCL Hospitals NIHR Biomedical Research Centre. Dr. Lumens has received a grant within the framework of the Dr. E. Dekker program of the Dutch Heart Foundation (NHS-2015T082). Funding Information: This work was supported by the Netherlands Cardiovascular Research Initiative, an initiative with support from the Dutch Heart Foundation (CVON2015-12 eDETECT). Dr. Asselbergs is supported by a Dekker scholarship (Junior Staff Member 2014T001, Dutch Heart Foundation) and UCL Hospitals NIHR Biomedical Research Centre. Dr. Lumens has received a grant within the framework of the Dr. E. Dekker program of the Dutch Heart Foundation (NHS-2015T082). Dr. Mast and Mr. Taha contributed equally to this work and are joint first authors. Publisher Copyright: © 2019 American College of Cardiology Foundation

PY - 2019/3

Y1 - 2019/3

N2 - OBJECTIVES The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols.BACKGROUND ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC.METHODS First-degree relatives of patients with ARVC were evaluated according to 2010 task force criteria, including RV deformation imaging (n = 128). Relatives fulfilling structural task force criteria were excluded for further analysis. At baseline, deformation patterns of the subtricuspid region were scored as type I (normal deformation), type II (delayed onset, decreased systolic peak, and post-systolic shortening), or type III (systolic stretching and large post-systolic shortening). The final study population comprised relatives who underwent a second evaluation during follow-up. Disease progression was defined as the development of a new 2010 task force criterion during follow-up that was absent at baseline.RESULTS Sixty-five relatives underwent a second evaluation after a mean follow-up period of 3.7 +/- 2.1 years. At baseline, 28 relatives (43%) had normal deformation (type I), and 37 relatives (57%) had abnormal deformation (type II or III) in the subtricuspid region. Disease progression occurred in 4% of the relatives with normal deformation at baseline and in 43% of the relatives with abnormal deformation at baseline (p <0.001). Positive and negative predictive values of abnormal deformation were, respectively, 43% (95% confidence interval: 27% to 61%) and 96% (95% confidence interval: 82% to 100%).CONCLUSIONS Normal RV deformation in the subtricuspid region is associated with absence of disease progression during nearly 4-year follow-up in relatives of patients with ARVC. Abnormal RV deformation seems to precede the established signs of ARVC. RV deformation imaging may potentially play an important role in ARVC family screening protocols. (C) 2019 by the American College of Cardiology Foundation.

AB - OBJECTIVES The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols.BACKGROUND ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC.METHODS First-degree relatives of patients with ARVC were evaluated according to 2010 task force criteria, including RV deformation imaging (n = 128). Relatives fulfilling structural task force criteria were excluded for further analysis. At baseline, deformation patterns of the subtricuspid region were scored as type I (normal deformation), type II (delayed onset, decreased systolic peak, and post-systolic shortening), or type III (systolic stretching and large post-systolic shortening). The final study population comprised relatives who underwent a second evaluation during follow-up. Disease progression was defined as the development of a new 2010 task force criterion during follow-up that was absent at baseline.RESULTS Sixty-five relatives underwent a second evaluation after a mean follow-up period of 3.7 +/- 2.1 years. At baseline, 28 relatives (43%) had normal deformation (type I), and 37 relatives (57%) had abnormal deformation (type II or III) in the subtricuspid region. Disease progression occurred in 4% of the relatives with normal deformation at baseline and in 43% of the relatives with abnormal deformation at baseline (p <0.001). Positive and negative predictive values of abnormal deformation were, respectively, 43% (95% confidence interval: 27% to 61%) and 96% (95% confidence interval: 82% to 100%).CONCLUSIONS Normal RV deformation in the subtricuspid region is associated with absence of disease progression during nearly 4-year follow-up in relatives of patients with ARVC. Abnormal RV deformation seems to precede the established signs of ARVC. RV deformation imaging may potentially play an important role in ARVC family screening protocols. (C) 2019 by the American College of Cardiology Foundation.

KW - arrhythmogenic right ventricular cardiomyopathy

KW - ARVD/C

KW - deformation imaging

KW - disease progression

KW - family screening

KW - strain imaging

KW - TASK-FORCE CRITERIA

KW - RISK STRATIFICATION

KW - DYSPLASIA/CARDIOMYOPATHY

KW - ECHOCARDIOGRAPHY

U2 - 10.1016/j.jcmg.2018.01.012

DO - 10.1016/j.jcmg.2018.01.012

M3 - Article

C2 - 29550307

SN - 1936-878X

VL - 12

SP - 446

EP - 455

JO - JACC-Cardiovascular Imaging

JF - JACC-Cardiovascular Imaging

IS - 3

ER -