Author + information
- Received September 18, 2017
- Revision received January 22, 2018
- Accepted January 23, 2018
- Published online March 4, 2019.
- Thomas P. Mast, MD, PhDa,b,∗,
- Karim Taha, BScc,∗,
- Maarten J. Cramer, MD, PhDa,
- Joost Lumens, PhDd,
- Jeroen F. van der Heijden, MD, PhDa,
- Berto J. Bouma, MD, PhDe,
- Maarten P. van den Berg, MD, PhDf,
- Folkert W. Asselbergs, MD, PhDa,g,h,
- Pieter A. Doevendans, MD, PhDa and
- Arco J. Teske, MD, PhDa,∗ ()
- aDepartment of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands
- bDepartment of Cardiology, Catharina Hospital Eindhoven, Eindhoven, the Netherlands
- cUniversity of Amsterdam, Amsterdam, the Netherlands
- dDepartment of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Maastricht, the Netherlands
- eDivision of Cardiology, Academic Medical Center Amsterdam, Amsterdam, the Netherlands
- fUniversity of Groningen, University Medical Center Groningen, Department of Cardiology, Groningen, the Netherlands
- gDurrer Center for Cardiovascular Research, ICIN-Netherlands Heart Institute, Utrecht, the Netherlands
- hInstitute of Cardiovascular Science, Faculty of Population Health Sciences, University College London, London, United Kingdom
- ↵∗Address for correspondence:
Dr. Arco J. Teske, Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, Heidelberglaan 100, 3584CX Utrecht, the Netherlands.
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.
- arrhythmogenic right ventricular cardiomyopathy
- deformation imaging
- disease progression
- family screening
- strain imaging
↵∗ Dr. Mast and Mr. Taha contributed equally to this work and are joint first authors.
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).
- Received September 18, 2017.
- Revision received January 22, 2018.
- Accepted January 23, 2018.
- 2019 American College of Cardiology Foundation
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