Impact of Prior Exercise Practice on Disease Characteristics in Desmoplakin-Related Arrhythmogenic Left Ventricular Cardiomyopathy.

Background: The harmful effects of high-intensity (HI) and long duration (LD) exercise have been reported in arrhythmogenic right ventricular cardiomyopathy, including in asymptomatic genetic variant carriers. However, the impact of exercise remains unclear in desmoplakin (DSP) gene variant carriers at risk of arrhythmogenic left ventricular cardiomyopathy (ALVC).

Objectives: The goal of this study was to assess the relationship between HI and LD exercise and the risk of developing an ALVC phenotype and its severity in DSP variant carriers.

Methods: In a monocentric retrospective study, patients with DSP pathogenic or likely pathogenic variant were interviewed about their exercise practice before genetic or disease diagnosis. HI and LD exercise were defined as having practiced at least one sport with an intensity ≥6 METs and ≥2.5 hours/week, respectively. We studied the association of HI and LD exercise practice with the risk of ALVC phenotype occurrence at diagnosis.

Results: A total of 85 patients were included (mean age 39 ± 19 years; 49% female). Sixty-nine percent had practiced HI exercise and 66% LD exercise. The rate of patients with an ALVC phenotype at time of diagnosis did not differ between patients having practiced HI and LD exercise and those who had not (71% vs 65% [P = 0.60] and 70% vs 69% [P = 0.95]). The distribution of prior calculated exercise dose was not different between patients with vs those without ALVC phenotype at diagnosis (P = 0.57).

Conclusions: Prior exercise intensity, duration, and cumulated lifetime dose were not associated with an increased risk of developing an ALVC phenotype at the time of diagnosis in DSP variant carriers.