The added value of abnormal regional myocardial function for risk prediction in arrhythmogenic right ventricular cardiomyopathy

Figures

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  Figure 1. Echocardiographic deformation imaging in an RV-focused view. (A) RVFWLS was calculated as the systolic peak strain from the averaged regional RV free wall deformation characteristic. (B) Based on predefined criteria, a division into three different deformation patterns was identified in all three segments of the RV lateral wall.15 Type I is normal deformation. Types II and III are abnormal deformation, whereby Type II is characterized by delayed onset of shortening, reduced systolic peak strain, and minor post-systolic shortening; Type III is characterized by little or no systolic peak strain, predominantly systolic stretching, and major post-systolic shortening. (C) For RV mechanical dispersion, a six-segment model of the RV was used, including both the lateral wall and the interventricular septum. It was calculated as the SD of the segmental time intervals from onset Q/R on the surface ECG to peak negative strain and expressed in ms.12 PV, pulmonic valve closure; RV, right ventricular.

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  Figure 2. Kaplan–Meier estimate of VA free survival for patients with ARVC without prior sustained VA. Dotted line represents cumulative 5-year survival. VA, ventricular arrhythmia.

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  Figure 3. VA free survival in presence of abnormal RV function measured by deformation imaging. (A) When dividing RVFWLS into three groups, a clear difference in VA-free survival was seen. (B) A normal RV deformation pattern (Type I) was associated with excellent negative predictive value (0.96, 95% CI 0.87–0.99). Especially the most abnormal Type 3 deformation was associated with worse outcome. RV, right ventricular; VA, ventricular arrhythmia.