Monitoring of Myocardial Involvement in ARVC | 113 Figure 5. Progression of myocardial disease substrates in the Digital Twin Progression of heterogeneity in estimated RV tissue properties between the three RV free wall segments, separated by age-group. A clear increase in heterogeneity was seen for all estimated RV tissue properties in the different age-groups. (Supplemental Table 2) DISCUSSION By monitoring myocardial disease progression in early ARVC, we found that LV GLS slightly deteriorated and especially RV deformation abnormalities progressed over time in all investigated age-groups. Digital Twins of the patient’s hearts were created to link deformation abnormalities to underlying myocardial disease substrates, revealing local differences in contractility, compliance and mechanical activation delay. Based on our findings, age-based tailoring of follow-up intervals would not be indicated. On the contrary, progression continued throughout all age-groups and was in multiple cases followed by sustained VA, indicating the need for lifelong follow-up. Progression of deformation abnormalities Our study showed that deformation imaging is a useful technique for monitoring of myocardial disease progression in ARVC. In a previous study on serial evaluation of ARVC relatives, one-third showed electrical progression during a 4-year follow-up and structural progression was rare.28 However, structural progression was measured by increase in 2010 TFC, which lack sensitivity for detection of early disease manifestation.17 We have previously shown that deformation imaging is superior for detection of early disease as compared to 2010 TFC.29 Deformation analyses in the current study showed that abnormalities were often present before fulfilment of structural 2010 TFC. Progression of abnormalities occurred in all three age-groups in both 6
RkJQdWJsaXNoZXIy MTk4NDMw