Monitoring of Myocardial Involvement in ARVC | 109 During follow-up, 6 patients experienced a first sustained VA, after a mean of 4.1 years (range 0.2 – 8.9 years). The mean age at event was 47 years (range 33 – 65 years). Progression of deformation abnormalities Both global (Figure 1) and segmental (Figure 2) deformation characteristics deteriorated during follow-up in all three age-groups. Deterioration in LVEF was not observed in any of the agegroups (-0.03% per year [95% CI -0.16 to 0.09]). However, LV function measured by LV GLS deteriorated slightly in all three age-groups by an average of 0.1%-point per year (95% CI 0.05 to 0.15%-point). Deterioration was more pronounced in the RV lateral wall in all agegroups, expressed by a mean worsening in RVFWLS of 0.6%-point per year (95% CI 0.46 to 0.70%-point). The three segmental deformation characteristics used to classify RV deformation type showed that the basal segment was most impaired in all age-groups, whereby an apex-tobase gradient was maintained during follow-up (Figure 2). When displaying progression of the deformation types during follow-up (Figure 3), the deformation pattern of the basal segment was most frequently impaired (type II or III). Progression to a more abnormal deformation pattern occurred in all age-groups, whereby the pattern in the basal segment deteriorated in about half of the cases and in the mid segment in about one third. Even in some subjects >50 years of age with normal RV deformation at first evaluation, progression towards abnormal deformation patterns was evident. Deformation in the apical segment was normal in most cases, and progression to an abnormal apical deformation pattern was rare. Yearly progression rates and mean values at inclusion and last follow-up are provided in Supplemental Table 1. Figure 2. Segmental myocardial disease progression Progression of segmental deformation parameters of the right ventricle (RV), separated by age-group. P-values are for progression during follow-up. The basal segment was most impaired in all age-groups, whereby an apex-to-base gradient was maintained during follow-up. (Supplemental Table 1) 6
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