Key result
Early ARVC shows progressive RV strain worsening of ~0.6 percentage points per year across all ages.
Why the study?
It was unknown whether repeated cardiac imaging follow-up protocols for early ARVC detection in at-risk relatives should be age-tailored.
Does myocardial disease progression in early ARVC differ across age groups (<30, 30-50, and ≥50 years)?
Cohort (n=82)
Single-blind
No
Does myocardial disease progression in early ARVC differ across age groups (<30, 30-50, and ≥50 years)?
Myocardial disease progression in early ARVC occurs at a similar rate across all age groups, challenging current guidelines by suggesting that clinical surveillance should not be discontinued in older patients.
Supports continued ARVC surveillance across ages; extends longitudinal strain data but leaves optimal intervals open pending larger cohorts.
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement of primarily the right ventricular myocardium, a substrate for life-threatening ventricular arrhythmias (VAs). 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: The goal of this study was 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 patients with early-stage ARVC and genotype-positive relatives without overt structural disease and VA at first evaluation into 3 groups: age <30 years, 30 to 50 years, and ≥50 years. 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 modeling framework. RESULTS: We included 313 echocardiographic assessments from 82 subjects (57% female, age 39 ± 17 years, 10% probands) during 6.7 ± 3.3 years of 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 right ventricular 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. CONCLUSIONS: Disease progression was similar in all age groups, and sustained VA also occurred in patients aged >50 years 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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Kirkels et al. (2023) conducted a cohort in Early arrhythmogenic right ventricular cardiomyopathy (ARVC) (n=82). Age group (younger vs middle-aged vs older) vs. Between age groups was evaluated on Right ventricular free wall longitudinal strain progression (0.6%-point per year worsening, 95% CI 0.46-0.70, p=<0.001). Myocardial disease progression in early ARVC occurred similarly across all age groups, with right ventricular longitudinal strain worsening by 0.6%-points per year, indicating lifelong follow-up.
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