16 | Chapter 1 While abnormal subtricuspid deformation patterns were not yet linked to arrhythmic outcome, researchers from Oslo did relate deformation abnormalities to arrhythmic events in the patients history.24,25 They reported strong correlations between RV and LV mechanical dispersion, measures of regional heterogeneity in contraction, and arrhythmic events. A combination of increased mechanical dispersion, T-wave inversions on the ECG, and a history of high intensity exercise was proposed to characterize a high risk profile regarding incidence of ventricular arrhythmias. Although many studies have confirmed the added value of deformation imaging, the technique is still not widely implemented in clinical practice. A possible contributor to this lagging implementation may be that many deformation methods have been used in single center studies without external validation. Second, most studies focus on deformation imaging as a single parameter, while added value should better be tested in a clinical practice based multimodality approach. In this thesis, ARVC is used as a disease model to demonstrate the road to clinical implementation of deformation imaging in genetic cardiomyopathies in general. It focusses both on detection and characterization of the early disease substrate as well as on arrhythmic risk stratification in patients and family-members at risk. Two major clinical challenges for clinicians which go hand in hand.
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