BIVENTRICULAR INVOLVEMENT IN ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

Background:Despite Arrhythmogenic right ventricular cardiomyopathy (ARVC) being a predominant right ventricle (RV) disease, concomitant left ventricle (LV) involvement has been recognized. Diagnosis is made through the 2010 Task force criteria (TFC), which are RV-centric, and has yet to include strain measurement. Our aim was to assess the utility of global longitudinal strain (GLS) of RV and LV for risk stratification of these patients.
Methods:
204 patients who met the TFC for the ARVC spectrum were included. Patients were categorized based on a cut-off of GLS -18% for impairment in both ventricles. Outcome was a composite of all-cause mortality, arrhythmic events, ICD therapy and heart failure.
Results:Patients (age 41 ± 17 years, 55% males) were divided into impaired, discordant (either RV or LV impaired), and normal strain groups. During a follow-up of 87 [24-136] months, 60 (29%) experienced the combined outcome, and a significant difference in event-free survival was observed (p<0.001) between the 3 groups (Fig). In the multivariate analysis, the GLS grouping remained associated with outcome (HR 2.398, 95% CI 1.065-5.395, p=0.040) after adjusting for age, gender, history of syncope and definite ARVC diagnosis. In a sub-analysis on definite and borderline ARVC patients, GLS grouping remained an independent predictor of events (p=0.027).
Conclusion: In ARVC patients, biventricular involvement by strain analysis has additional prognostic value, with outcomes differing if either the RV, LV or both are affected.