Exercise and phenotypic progression in arrhythmogenic cardiomyopathy

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Abstract Background Intense exercise may exacerbate disease expression in arrhythmogenic cardiomyopathy (ACM). Natural history and phenotypic progression of ACM is still poorly understood. Purpose The aim of the study was to investigate the impact of exercise on phenotypic progression in patients with ACM, focusing on cardiovascular magnetic resonance (CMR) features. Methods A retrospective analysis of clinical characteristics of patients with ACM seen was conducted. Patients with at least two sequential CMR scans were included and categorized in two groups according to the levels of exercise activity after diagnosis: "sedentary/mild exercise" and "moderate/intense exercise" group. Morphological and functional parameters at CMR were assessed longitudinally. Myocardial fibrosis (MF) progression was defined as the development of de novo fibrosis or an involvement of at least two additional myocardial segments from baseline. Result A total of 152 patients were investigated with at least one CMR. Of these, 60 (46% males, mean age 41 ± 16 years) patients were assessed with at least 2 CMRs (mean interval between scans was 3.7 ± 3.2 years). A pathogenic (P) or likely pathogenic (LP) variant was found in 50% of cases (PKP2: 30%, FLNC: 10%, other genes: 10%). Across the cohort, left ventricular (LVEF) and right ventricular (RVEF) ejection fraction declined significantly (59.2 ± 9.1% vs 57.0 ± 8.9%, p = 0.025; 54.3 ± 10.7% vs 52.1 ± 9.7%, p = 0.014, respectively). Nine patients with no evidence of MF at the initial CMR developed MF at follow-up (Figure 1). Overall, MF progression was observed in 36% of the cohort. The "moderate-intense exercise" group (n = 32) exhibited greater increases in indexed left ventricular end-diastolic volume (LVEDVi) and indexed right ventricular end-diastolic volume (RVEDVi) compared to the "sedentary/mild exercise" group (n = 28) (LVEDVi from 86.0 ± 18.4 to 92.6 ± 15.4 ml/m², vs 86.4 ± 21.2 to 82.5 ± 15.4 ml/m², p = 0.004; RVEDVi from 90.1 ± 20.9 ml/m² to 96.3 ± 18.6 ml/m² vs. 92.5 ± 24.7 ml/m² to 90.8 ± 22.7 ml/m², p = 0.027). MF progression was similar across groups (33% of patients in "sedentary/mild exercise" group vs 40% of patients in the "moderate/intense exercise" group, p = 0.690). Among PKP2 P/LP variants carriers (n = 18), a more pronounced reduction in RVEF was observed in the "moderate/intense exercise" group (61.3 ± 9.7% to 56.5 ± 9.2%) compared to the "sedentary/mild exercise" (from 50.2 ± 13.1% to 51.5 ± 11.4%), p = 0.041. Conclusion Structural and functional phenotypic progression in the short term (3.5 years) is common in patients with ACM, emphasizing the need for ongoing surveillance. Myocardial fibrosis progresses slightly more often in patients who engage in moderate and intense regular exercise. In PKP2 carriers, moderate/intense exercise appears to be associated with a decline in right ventricular systolic function.Changes in CMR parameters between scansMyocardial fibrosis progression

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