{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,24]],"date-time":"2025-09-24T09:46:35Z","timestamp":1758707195916,"version":"3.37.3"},"reference-count":24,"publisher":"Oxford University Press (OUP)","issue":"Supplement_1","license":[{"start":{"date-parts":[[2021,3,5]],"date-time":"2021-03-05T00:00:00Z","timestamp":1614902400000},"content-version":"vor","delay-in-days":4,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003246","name":"Netherlands Organisation for Scientific Research","doi-asserted-by":"publisher","award":["016.176.340"],"award-info":[{"award-number":["016.176.340"]}],"id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002996","name":"Dutch Heart Foundation","doi-asserted-by":"crossref","award":["2018T094"],"award-info":[{"award-number":["2018T094"]}],"id":[{"id":"10.13039\/501100002996","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Dr Dekker Program","award":["2015T082"],"award-info":[{"award-number":["2015T082"]}]},{"name":"Theo-Rossi di Montelera (TRM) foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,3,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Arrhythmogenic cardiomyopathy (AC) is an inherited cardiac disease, characterized by life-threatening ventricular arrhythmias and progressive cardiac dysfunction. The aim of this study is to use computer simulations to non-invasively estimate the individual patient\u2019s myocardial tissue substrates underlying regional right ventricular (RV) deformation abnormalities in a cohort of AC mutation carriers.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>In 68 AC mutation carriers and 20 control subjects, regional longitudinal deformation patterns of the RV free wall (RVfw), interventricular septum (IVS), and left ventricular free wall (LVfw) were obtained using speckle-tracking echocardiography. We developed and used a patient-specific parameter estimation protocol based on the multi-scale CircAdapt cardiovascular system model to create virtual AC subjects. Using the individual\u2019s deformation data as model input, this protocol automatically estimated regional RVfw and global IVS and LVfw tissue properties. The computational model was able to reproduce clinically measured regional deformation patterns for all subjects, with highly reproducible parameter estimations. Simulations revealed that regional RVfw heterogeneity of both contractile function and compliance were increased in subjects with clinically advanced disease compared to mutation carriers without clinically established disease (17\u2009\u00b1\u200913% vs. 8\u2009\u00b1\u20094%, P\u2009=\u20090.01 and 18\u2009\u00b1\u200911% vs. 10\u2009\u00b1\u20097%, P\u2009&amp;lt;\u20090.01, respectively). No significant difference in activation delay was found.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Regional RV deformation abnormalities in AC mutation carriers were related to reduced regional contractile function and tissue compliance. In clinically advanced disease stages, a characteristic apex-to-base heterogeneity of tissue abnormalities was present in the majority of the subjects, with most pronounced disease in the basal region of the RVfw.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/europace\/euaa407","type":"journal-article","created":{"date-parts":[[2020,12,14]],"date-time":"2020-12-14T16:35:16Z","timestamp":1607963716000},"page":"i153-i160","source":"Crossref","is-referenced-by-count":11,"title":["Electromechanical substrate characterization in arrhythmogenic cardiomyopathy using imaging-based patient-specific computer simulations"],"prefix":"10.1093","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8927-9110","authenticated-orcid":false,"given":"Nick","family":"van Osta","sequence":"first","affiliation":[{"name":"Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50 (UNS50), 6229 ER Maastricht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Feddo","family":"Kirkels","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50 (UNS50), 6229 ER Maastricht, The Netherlands"},{"name":"Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6019-7376","authenticated-orcid":false,"given":"Aurore","family":"Lyon","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50 (UNS50), 6229 ER Maastricht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tijmen","family":"Koopsen","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50 (UNS50), 6229 ER Maastricht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tim","family":"van Loon","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50 (UNS50), 6229 ER Maastricht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maarten-Jan","family":"Cramer","sequence":"additional","affiliation":[{"name":"Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arco J","family":"Teske","sequence":"additional","affiliation":[{"name":"Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht, The 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