Thomas P Mast1, Karim Taha2, Maarten J Cramer3, Joost Lumens4, Jeroen F van der Heijden3, Berto J Bouma5, Maarten P van den Berg6, Folkert W Asselbergs7, Pieter A Doevendans3, Arco J Teske8. 1. Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands; Department of Cardiology, Catharina Hospital Eindhoven, Eindhoven, the Netherlands. 2. University of Amsterdam, Amsterdam, the Netherlands. 3. Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands. 4. Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Maastricht, the Netherlands. 5. Division of Cardiology, Academic Medical Center Amsterdam, Amsterdam, the Netherlands. 6. University of Groningen, University Medical Center Groningen, Department of Cardiology, Groningen, the Netherlands. 7. Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands; Durrer Center for Cardiovascular Research, ICIN-Netherlands Heart Institute, Utrecht, the Netherlands; Institute of Cardiovascular Science, Faculty of Population Health Sciences, University College London, London, United Kingdom. 8. Department of Cardiology, Division Heart and Lungs, University Medical Center Utrecht, Utrecht, the Netherlands. Electronic address: a.j.teske-2@umcutrecht.nl.
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.
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.
Authors: Marijn H A Groen; Laurens P Bosman; Arco J Teske; Thomas P Mast; Karim Taha; Frebus J Van Slochteren; Maarten J Cramer; Pieter A Doevendans; René van Es Journal: Echocardiography Date: 2020-05-03 Impact factor: 1.724
Authors: Nick van Osta; Aurore Lyon; Feddo Kirkels; Tijmen Koopsen; Tim van Loon; Maarten J Cramer; Arco J Teske; Tammo Delhaas; Wouter Huberts; Joost Lumens Journal: Philos Trans A Math Phys Eng Sci Date: 2020-05-25 Impact factor: 4.226
Authors: Karim Taha; Mimount Bourfiss; Anneline S J M Te Riele; Maarten-Jan M Cramer; Jeroen F van der Heijden; Folkert W Asselbergs; Birgitta K Velthuis; Arco J Teske Journal: Eur Heart J Cardiovasc Imaging Date: 2021-07-20 Impact factor: 6.875
Authors: Halszka Kamińska; Łukasz A Małek; Marzena Barczuk-Falęcka; Marta Bartoszek; Ewa Strzałkowska-Kominiak; Mikołaj Marszałek; Ewa Brzezik; Michał Brzewski; Bożena Werner Journal: J Clin Med Date: 2021-03-24 Impact factor: 4.241