104 | Chapter 6 ABSTRACT Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement of primarily the right ventricular (RV) myocardium, a substrate for life-threatening ventricular arrhythmias (VA). Repeated cardiac imaging of at-risk relatives is important for early disease detection. However, it is not known whether screening should be age-tailored. Objectives To assess the need for age-tailoring of follow-up protocols in early ARVC by evaluating myocardial disease progression in different age-groups. Methods We divided early-stage ARVC patients and genotype positive relatives without overt structural disease and VA at first evaluation into three groups: <30, 30-50 and ≥50 years old. Longitudinal biventricular deformation characteristics were used to monitor disease progression. To link deformation abnormalities to underlying myocardial disease substrates, Digital Twins were created using an imaging-based computational modelling framework. Results We included 313 echocardiographic assessments from 82 subjects (57% female, age 39±17 years, 10% probands) during 6.7±3.3 years follow-up. Left ventricular global longitudinal strain slightly deteriorated similarly in all age-groups (0.1%-point per year [95% CI 0.05-0.15]). Disease progression in all age-groups was more pronounced in the RV lateral wall, expressed by worsening in longitudinal strain (0.6%-point per year [95% CI 0.46-0.70]) and local differences in myocardial contractility, compliance and activation delay in the Digital Twin. Six patients experienced VA during follow-up. Conclusion Disease progression was similar in all age-groups and sustained VA also occurred in patients >50 years old without overt ARVC phenotype at first evaluation. Unlike recommended by current guidelines, our study suggests that follow-up of ARVC patients and relatives should not stop at older age.
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