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Electromechanical substrate characterization in arrhythmogenic cardiomyopathy using imaging-based patient-specific
Nick van Osta1, Feddo Kirkels1,2, Aurore Lyon1
1Department of Biomedical Engineering, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50 (UNS50), 6229 ER Maastricht, The Netherlands.
Summary
Computer simulations non-invasively estimated myocardial tissue substrates in arrhythmogenic cardiomyopathy (AC) patients. AC mutation carriers with advanced disease showed increased RV heterogeneity in contractile function and compliance.
Area of Science:
- Cardiology
- Biomedical Engineering
- Computational Biology
Background:
- Arrhythmogenic cardiomyopathy (AC) is an inherited cardiac disease causing arrhythmias and heart dysfunction.
- Understanding the underlying myocardial tissue substrates is crucial for AC management.
Purpose of the Study:
- To non-invasively estimate individual patient myocardial tissue substrates in AC mutation carriers.
- To correlate regional right ventricular (RV) deformation abnormalities with specific tissue properties.
Main Methods:
- Used speckle-tracking echocardiography to obtain RV deformation patterns in 68 AC mutation carriers and 20 controls.
- Developed a patient-specific computational model (CircAdapt) to estimate RV and LV tissue properties.
- Validated the model's ability to reproduce clinical deformation data.
Main Results:
- Simulations accurately reproduced regional deformation patterns.
- Increased RV free wall (RVfw) heterogeneity in contractile function and compliance was observed in advanced AC.
- No significant difference in activation delay was found between disease stages.
Conclusions:
- Regional RV deformation abnormalities in AC are linked to reduced contractile function and tissue compliance.
- Advanced AC stages exhibit apex-to-base heterogeneity in RVfw tissue abnormalities, predominantly in the basal region.

