108 | Chapter 6 Table 1. Baseline characteristics of 82 ARVC patients and family members, comparing three different age-groups total (n = 82) Age <30 (n = 27) Age 30-50 (n = 32) Age ≥50 (n = 23) p-value Age (years) 39 ± 17 20 ± 6 39 ± 6 60 ± 7 Female sex, n (%) 47 (57%) 15 (56%) 20 (63%) 12 (52%) 0.723 Proband, n (%) 8 (10%) 0 (0%) 4 (13%) 4 (17%) 0.064 Follow-up time (years) 6.5 ± 3.1 7.1 ± 2.9 6.9 ± 3.5 5.2 ± 2.5 0.109 VA during follow-up, n (%) 6 (7%) 0 (0%) 4 (13%) 2 (9%) 0.197 (Likely-)pathogenic variant, n (%) 75 (92%) 27 (100%) 29 (91%) 19 (83%) 0.077 PKP2, n (%) 69 (84%) 24 (89%) 28 (88%) 17 (74%) 0.326 DSG, n (%) 5 (6%) 2 (7%) 1 (3%) 2 (9%) 0.616 DSP, n (%) 1 (1%) 1 (4%) 0 (0%) 0 (0%) 0.610 Values are mean ± SD, median (IQR), or frequencies (%). Abbreviations: DSG2, desmoglein-2 gene; DSP, desmoplakin gene; PKP2, plakophilin-2 gene; VA, life-threatening ventricular arrhythmia. Figure 1. Global myocardial disease progression Progression of left ventricular (LV) ejection fraction (LVEF), LV global longitudinal strain (LV GLS), and right ventricular free wall longitudinal strain (RVFWLS), separated by age-group. P-values are for progression during follow-up. Deterioration in LVEF was not observed in any of the age-groups, but LV function deteriorated by absolute 0.1%-point per year (95% CI 0.05 to 0.15%-point) worsening of GLS. Deterioration was faster in the RV lateral wall, expressed by a mean worsening of absolute 0.6%-point per year (95% CI 0.46 to 0.70%-point). (Supplemental Table 1)
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