Key result
In carriers of the PLN p.(Arg14del) variant, the amount of exercise prior to clinical presentation was not associated with the development of sustained ventricular arrhythmias (18.3% vs 18.4%, p=0.974).
Why the study?
While exercise is linked to earlier onset of ARVC and ventricular arrhythmias in desmosomal variant carriers, the effect of exercise in PLN p.(Arg14del) carriers was unknown.
Does a higher volume of exercise increase the risk of developing ARVC, DCM, ventricular arrhythmias, or heart failure in PLN p.(Arg14del) carriers?
Cohort (n=207)
Yes
Does a higher volume of exercise increase the risk of developing ARVC, DCM, ventricular arrhythmias, or heart failure in PLN p.(Arg14del) carriers?
In PLN p.(Arg14del) carriers, mild-to-moderate exercise is not associated with an increased risk of developing ventricular arrhythmias, heart failure, or cardiomyopathy, suggesting exercise restrictions may not be necessary for asymptomatic carriers.
May not warrant restricting mild-moderate exercise in asymptomatic PLN p.(Arg14del) carriers; challenges desmosomal ARVC data and leaves open prospective confirmation.
BACKGROUND: Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. METHODS: In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. RESULTS: Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. CONCLUSION: There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients.
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Lint et al. (2023) conducted a cohort in PLN p.(Arg14del) cardiomyopathy (n=207). Exercise vs. Lower levels of exercise (least active 50%) was evaluated on Sustained ventricular arrhythmias (p=0.974). In carriers of the PLN p.(Arg14del) variant, the amount of exercise prior to clinical presentation was not associated with the development of sustained ventricular arrhythmias (18.3% vs 18.4%, p=0.974).
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