Association of Echocardiographic Parameters of Right Ventricular Remodeling and Myocardial Performance with Modified Task Force Criteria in Adolescents with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Objectives: The usefulness of echo parameters including those used in the Modified task force criteria (MTFC) and of others such as strain imaging to diagnose Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) in adolescence is not well established. We examined the association of MTFC echocardiographic criteria and of RV longitudinal 2-D strain with a diagnosis of ARVC in adolescents.

Methods: This is a single-center study. Echocardiograms from 120 adolescent ARVC patients (13 ± 4 years, BSA 1.53 ± 0.38), who were referred to our tertiary center were retrospectively analyzed. According to MTFC criteria, patients were classified into definite (n = 38), borderline ARVC (n = 39) and possible (n = 43) ARVC. Results were compared with a control cohort (n = 35). Echocardiographic MTFC parameters and additionally, pulsed-wave tissue Doppler Imaging parameters as well as mean and segmental RV longitudinal peak systolic strain were measured and their disease association analyzed.

Results: Some BSA indexed MTFC parameters as well as RV end-diastolic diameters were significantly different between patients and controls and between ARVC sub-groups. Mean (Sl) and segmental RV peak longitudinal peak systolic strain were significantly different between controls (Sl-25 ± 3) and patients with definite (Sl-21 ± 4) ARVC, and also among disease sub-groups. Importantly, multivariate risk analysis showed that RV Sl was significantly associated with ARVC diagnosis and its severity (multivariate OR [95% CI] = 1.228 [1.103, 1.366], p < 0.001). The other RV echocardiographic parameter being associated with disease was RV end diastolic diameter at apical third of RV (multivariate OR [95% CI] =1.511 [1.329, 1.718], p < 0.001).

Conclusion: MTFC echocardiographic criteria are significantly different between ARVC patients and controls and between the different diagnostic sub-groups; allowing differentiation between patients and controls and between different diagnostic sub-groups. However, in our to date largest cohort, absolute current echocardiographic MTFC are not met by the majority of adolescent ARVC patients, particularly when indexed to BSA, suggesting a need for adolescent specific cut-off values for echocardiographic parameters in the diagnosis of ARVC.