Abstract

Highlights•Impaired strain can identify both right and left ventricular involvement in ARVC•Biventricular strain assessment is crucial as patients can present LV involvement only•Patients can be grouped into normal, discordant (LV or RV) or impaired (both) strain•Involvement of one or both ventricles is associated with mortality and arrhythmias•Biventricular strain impairment has prognostic value across ARVC diagnosis spectrumAbstractDespite arrhythmogenic right ventricular cardiomyopathy (ARVC) being predominantly a right ventricular (RV) disease, concomitant left ventricular (LV) involvement has been recognized. ARVC is diagnosed by the RV-centric 2010 Task Force Criteria(TFC) using routine echocardiography, but previous studies have suggested that strain imaging may be more sensitive to detect RV and LV dysfunction. No data however are available regarding the additional value of combining biventricular strain for risk stratification. This study aims to assess the prognostic value of both LV global longitudinal strain (GLS) and RV free wall strain (FWLS) in patients with ARVC. To accomplish this, 204 patients who met the TFC for the ARVC spectrum were included. Patients (age 41±17 years,55% men) were divided into impaired(n=33), discordant (RV or LV impaired, n=70), and normal (n=101) strain groups based on a value of ≥18% for both ventricles. During a follow-up of 87 [24-136] months, 57 (28%) experienced the composite outcome of all-cause mortality, arrhythmic events, implantable cardioverter defibrillator therapy and heart failure events, and a significant difference in event-free survival was observed (p<0.001) between the 3 groups. In the multivariable analysis, the strain groups remained associated with outcomes (p=0.014) after adjusting for age, sex, history of syncope and definite ARVC diagnosis. A sub-analysis including only definite and borderline diagnosed ARVC confirmed that the strain groups were independently predictive of the endpoint (p=0.023). In conclusion, biventricular involvement by strain analysis may help risk stratification in ARVC patients, with the worst outcomes of patients with both RV and LV impaired strain.Graphical abstractThe upper panel shows the RV and LV strain measurements (RV free wall strain and LV global longitudinal strain) performed to define the strain groups based on the relative ventricular function and with the cut-off value of 18%: impaired strain, discordant and normal strain. Lower panel depicts the difference in outcome between the strain groups based on Kaplan-Meier analysis (left) and the incremental value of using strain groups in assessing prognosis in addition to age, sex and ARVC diagnosis, as compared to solely using LV or RV strain (right). ARVC, Arrhythmogenic Right Ventricular Cardiomyopathy; FWLS, right ventricular free wall longitudinal strain; LV, Left ventricle; LV GLS, left ventricular global longitudinal strain; RV, Right ventricle.