116 | Chapter 6 Central Illustration. Monitoring age-related penetrance in ARVC Patients with early arrhythmogenic right ventricular cardiomyopathy (ARVC) and genotype positive family members are frequently screened for structural abnormalities which are strongly associated with life-threatening ventricular arrhythmias. To find out whether this extensive screening can be stopped at older age, we performed biventricular deformation analyses in 313 longitudinal echocardiograms of 82 early ARVC patients and family members at risk divided over three age-groups. Digital Twins of the patients’ hearts were created to reveal the local tissue substrates underlying myocardial deformation abnormalities. We found similar patterns of progression in both young, middle-aged and older subjects and first life-threatening arrhythmias still occurred in patients >50 years old, suggesting that follow-up of early ARVC patients and family members should not stop at older age.
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