General Introduction and Thesis Outline | 19 over two decades, the technique is still not widely implemented in clinical practice. An important step towards wide implementation could be inclusion in clinical guidelines, which requires convincing evidence of the added value of deformation imaging. One important strength of deformation imaging is that it is a more sensitive method for detection of early disease manifestation compared to conventional global imaging parameters. In Chapter 7, we summarized all available evidence regarding the value of deformation imaging in early detection of genetic cardiomyopathies in relatives. While the value of deformation imaging as a stand-alone index has been extensively published, there is a lack of clinical practice based multi-modality studies. Inclusion in clinical guidelines requires evidence of added value in a real-world setting. In other words, it should improve current clinical practice. In Chapter 8 we used ARVC as a model to investigate added value of deformation imaging for arrhythmic risk prediction in a multimodality approach. We integrated deformation imaging with the validated ARVC risk calculator17 to test added value in a clinical practice based approach. Addendum: left-sided wall stress causing arrhythmia In Chapter 10 we provide an addendum to this thesis. Where the subtricuspid region is a weak spot in the RV, the submitral region can be a similar weak spot in the LV. In both cases, the hypothesis is that high mechanical stress on a thin wall can cause life-threatening arrhythmias. Dedicated imaging may help to identify these weak spots. On the right side, ARVC is an important contributor to this weak spot (desmosomal fragility). On the left side, mitral annular disjunction (MAD), defined as atrial displacement of the mitral valve hinge point may cause a weak spot (Figure 4). In the affected area, the thin atrial wall has to withstand the high wall stress which is normally applied to the thick LV wall. This condition recently received increasing attention in relation to unexplained ventricular arrhythmia.29,30 In this chapter, we reanalyzed cardiac magnetic resonance images of patients who survived an unexplained cardiac arrest with special attention to the mitral valve area. Figure 4. Mitral annular disjunction Mitral annular disjunction (MAD) and mitral valve prolapse at end-systole in the longitudinal 3-chamber view on cardiac magnetic resonance imaging (left panel) and in a schematic overview (right panel). The orange arrow indicates the longitudinal MAD distance along the atrial wall. The dashed line connects the annular hinge points and represents the annular plane. The dotted line perpendicular on the annular plane measures the mitral valve prolapse. LA = left atrium, LV = left ventricle. 1
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