44 | Chapter 3 Limitations This study had a cross-sectional design with retrospective outcome adjudication with inherent limitations. The predictive value of the investigated parameters should be assessed in a prospective trial. RV deformation imaging reflects partly the same RV functional status as other echocardiographic and CMR indices and a multimodality approach will probably offer the best results. To investigate the clinical impact of RV deformation measurements, the additive predictive value should be explored with regard to the existing risk calculation model.30 A single observer performed all deformation analyses in this study. We considered this a strength for the external validation of the methodology. Although our inter-observer variability analysis showed excellent agreement, this approach may represent a limitation for the clinical external validation. Subjects evaluated at higher age were more likely to have experienced an event. However, also when adjusted for age, RV deformation abnormalities were associated with substantial increase in odds for history of VA. Implantation of ICDs caused a risk of bias for detection of arrhythmic events, but due to the low number of appropriate ICD therapies this risk is considered to be low in the current study. Data from family members may not be independent. However, since we included patients from 86 different families, we considered it unlikely that overall results were influenced by dependence within single families. A comparison with healthy controls was not performed in this study, but can be found for RV deformation patterns6 and mechanical dispersion8 separately in previous studies. The majority of patients had PKP2 mutations which may limit the extrapolation to other genetic causes of AC.
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