Feddo Kirkels

112 | Chapter 6 Figure 4. Myocardial disease progression in two patients with early ARVC Panel A, case study 1. This 20-year-old patient was followed in the outpatient department during 4.3 years. At first evaluation, RV deformation was slightly abnormal in the basal segment. During follow-up, the deformation pattern of especially the basal segment and to a lesser extent the mid segment became increasingly abnormal with strongly reduced peak strain. In the Digital Twin, it was estimated that the heterogeneity relied on mostly reduced contractility and a little reduced compliance. Panel B, case study 2. This 36-year-old patient experienced a sustained ventricular arrhythmia 2.9 years after the first evaluation (yellow lightning bolt). During the first examination, RV deformation was slightly abnormal in the basal segment, with delayed onset of shortening, slightly reduced peak strain and mild post-systolic shortening. During follow-up, deformation patterns became increasingly abnormal in all RV segments, whereby the basal pattern was most affected (black panels). The apex-to-base heterogeneity was also expressed in the estimated RV tissue properties in the Digital Twin (panels on the right). Heterogeneity was first observed in the mechanical activation delay and later also in contractility and compliance.

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