Right ventricular scalloping index as CMR-derived marker for diagnosis of arrhythmogenic right ventricular cardiomyopathy

Abstract

Background: The cardiac magnetic resonance (CMR) evaluation of right ventricular (RV) morphologic abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is subjective. Here we aimed to use a quantitative index, the right ventricular scalloping index (RVSI), to standardize the measurement of RV free wall scalloping and aid in the imaging diagnosis. Methods: We retrospectively included 15 patients with definite ARVC and 45 age- and sex-matched patients with idiopathic right ventricular outflow tract ventricular arrhythmia (RVOT-VA) as controls. The RVSI was measured from cine images on four-chamber view to evaluate its ability to distinguish between ARVC and RVOT-VA patients. Other cardiac functional parameters including strain analysis were also performed. Results: The RVSI was significantly higher in the ARVC than RVOT-VA group (1.56±0.23 vs. 1.30±0.08, p<0.001). The diagnostic performance of the RVSI was superior to the RV global longitudinal, circumferential, and radial strains, RV ejection fraction, and RV end-diastolic volume index. The RVSI demonstrated high intra- and interobserver reliability (intraclass correlation coefficient, 0.94 and 0.96, respectively). RVSI was a strong discriminator between ARVC and RVOT-VA patients (Area under curve [AUC], 0.91; 95% confidence interval [CI], 0.82–0.99). A cut-off value of RVSI ≥ 1.49 provided an accuracy of 90.0%, specificity of 97.8%, sensitivity of 66.7%, positive predictive value (PPV) of 90.9%, and a negative predictive value (NPV) of 89.8%. In a multivariable analysis, a family history of ARVC or sudden cardiac death (odds ratio, 38.71; 95% confidence interval, 1.48–1011.05; p=0.028) and an RVSI ≥ 1.49 (odds ratio, 64.72; 95% confidence interval, 4.58–914.63; p=0.002) remained predictive of definite ARVC. Conclusion: RVSI is a quantitative method with good performance for the diagnosis of definite ARVC.