Feddo Kirkels

138 | Chapter 7 low. To investigate the clinical significance of deformation imaging in relatives with regard to hard end-points, larger studies with longer follow-up are needed. Due to the heterogeneity in definitions and methods between the studies, we could not perform a meta-analysis, nor could we extract cut-off values from the published data. Finally, when applying these results into clinical practice, it should be taken into account that different software packages were used among the different studies. While the inter-vendor differences for global measurements are small,17 there is still considerable variability in the detection of regional functional abnormalities among different vendors.63 CONCLUSION Deformation imaging can unmask early signs of disease in relatives of DCM, HCM and ACM patients. The main observations in these relatives are (1) reduced GLS in relatives at risk for DCM, (2) reduced basal (antero)septal strain in relatives at risk for HCM, and (3) reduced RV free wall strain in relatives at risk for ACM. Considering the prognostic value in early stages of DCM and ACM, we recommend routine measurement of disease-specific deformation parameters in these relatives. Collaborations between research groups are needed to create opportunities for larger studies and to create a platform for external validation.

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