Feddo Kirkels

Early Detection of Genetic Cardiomyopathies in Relatives | 147 Global deformation parameters Regional deformation parameters Control group Main result(s) GLS, LVMD, RVMD, RVFWLS none N/A 4 relatives with VA during follow-up. LVMD higher and RVFWLS lower in these relatives GLS, LVMD, RVGLS (6 segments), RVMD None N/A 15 subjects with arrhythmic events. RVMD lower in subjects with arrhythmic events (P = 0.003). GLS, LVMD None N/A 25 relatives had structural progression during follow-up. Higher LVMD at baseline predicted structural progression in relatives (P = 0.02). none Basal RV deformation pattern N/A Basal RV deformation patterns have high negative predictive value for disease progression during 3.7 ± 2.1 years follow-up none Basal, mid, apical RV deformation pattern N/A Basal RV deformation patterns reveal disease progression during 6.6 ± 3.1 years follow-up, conventional measurements remain unchanged none Basal, mid, apical RV electromechanical interval 30 healthy volunteers without history of heart disease Electromechanical interval prolonged in relatives in RV basal area (P<0.001). Prolonged electromechanical interval associated with arrhythmic outcome during 4.2 ± 3.1 years follow-up GLS, LVMD, RVFWLS, RVMD none 10 genotype-negative relatives and 30 healthy volunteers All parameters impaired in relatives (P<0.001). RVMD strongest association with arrhythmias. 7

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