Monitoring of Myocardial Involvement in ARVC | 117 CONCLUSION ARVC disease progressed similarly in all age-groups of patients presenting with early ARVC disease during 7 years of follow-up. We created Digital Twins of the patients’ hearts to link deformation abnormalities to underlying myocardial disease substrates, revealing local differences in contractility, compliance and mechanical activation delay. Potentially lifethreatening arrhythmias also occurred in patients >50 years old without overt ARVC phenotype at first evaluation, with the oldest patient aged 65 years. Contradicting previously published statements, our study suggests that follow-up of ARVC patients and family members should not stop at older age. CLINICAL PERSPECTIVES Competency in Patient Care and Procedural Skills In patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) fibrofatty replacement of right ventricular myocardium progresses similarly across the age spectrum, and the initial presentation of life-threatening arrhythmias may emerge at any age, suggesting that clinical surveillance of patients with ARVC and their family members should not stop at older age. Translational Outlook The use of cascade genetic screening is exposing an expanding population at risk of ARVC. Echocardiographic deformation imaging is useful for monitoring of disease progression. Insight into the evolution of tissue substrates of individual patients may improve prediction of adverse outcomes. 6
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