Journal
EUROPACE
Volume 23, Issue -, Pages I153-I160Publisher
OXFORD UNIV PRESS
DOI: 10.1093/europace/euaa407
Keywords
Arrhythmogenic right ventricular cardiomyopathy; Computer modelling and simulation; Deformation imaging; Contractility; Right ventricle
Categories
Funding
- Netherlands Organisation for Scientific Research (NWO-ZonMw, VIDI grant) [016.176.340]
- Dutch Heart Foundation (ERA-CVD JTC2018 grant) [2018T094, 2015T082]
- Theo-Rossi di Montelera (TRM) foundation
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The study used computer simulations to assess myocardial tissue substrates in AC mutation carriers, finding that in clinically advanced disease stages, regional RV deformation abnormalities were associated with reduced contractile function and tissue compliance.
Aims 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's myocardial tissue substrates underlying regional right ventricular (RV) deformation abnormalities in a cohort of AC mutation carriers. Methods and results 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 multiscale CircAdapt cardiovascular system model to create virtual AC subjects. Using the individual's 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 +/- 13% vs. 8 +/- 4%, P=0.01 and 18 +/- 11% vs. 10 +/- 7%, P <0.01, respectively). No significant difference in activation delay was found. Conclusion 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.
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