Abstract

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable cardiomyopathy characterized by right ventricular (RV) myocardium dysfunction leading to RV failure and fatal arrhythmias. Hypothesis: RV global longitudinal strain (GLS) derived from two-dimensional speckle-tracking echocardiography (2DSTE) contributes to diagnostic and prognostic assessment in ARVC. Methods: We retrospectively evaluated the echocardiographic data of 67 patients referred with suspected ARVC and 27 age- and gender-matched healthy controls (Group C). Of 67 suspected ARVC patients, 34 patients (Group A) were diagnosed as ARVC, while remaining 33 patients (Group B) were not diagnosed due to not meeting criteria. Tricuspid annular plane systolic excursion (TAPSE), RV end-diastolic and end-systolic area (RVEDA, RVESA), RV fractional area change (RVFAC) and GLS were measured to assess the RV function. Outcome was assessed according to death and hospitalization with heart failure, fatal arrhythmia and appropriate defibrillator shock. Results: Patients in Group A had significantly reduced values of TAPSE, RVFAC and GLS, and significantly greater vales of RVEDA and RVESA in comparison among 3 groups. In Group A, 11 adverse cardiac events were observed during averaged 718 follow-up days. Multivariate Cox’s regression analysis showed that GLS was an independent prognostic factor. Kaplan-Meier survival analysis revealed there was a significant difference in outcome between patient groups divided by the median value (11%) of GLS in Group A. Conclusions: GLS in RV may be a tool to assist in diagnosis of ARVC, and be a superior prognostic factor in ARVC patients.

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