Arrythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by progressive fibrofatty replacement of the right ventricle (RV). The diagnosis of ARVC remains a challenge for clinicians. We hypothesized that 2D strain imaging helps to differentiate ARVC from normals.

20 patients meeting both the Task Force and cardiac MRI criteria for ARVC were compared to 10 age-matched controls. From the apical-4 chamber view, we measured the end-diastolic RV area, the peak systolic S tissue velocity at the tricuspid level. A 2D strain region of interest including the RV free wall and the septum (occasionally diseased in ARVC) was manually traced, from which the averaged peak systolic 2D strain along 6 segments was calculated. Pulmonary systolic pressure as well as the systolic right atrial area were measured as surrogate for RV loading conditions. Overall, patients with ARVC had both larger right atrial and ventricular area (p<0.03 and p<0.02, respectively), compared to controls while pulmonary systolic pressure were similar in both groups (p=0.40). Peak systolic 2D strain was statistically deteriorated in ARCV patients (13±6 vs. 23±4% in normals, p<0.001) so as peak systolic S tissue velocity (8±3 vs. 12±4 in normals, p<0.005). From the ROC analysis, a peak systolic 2D strain of 19.7% had a sensitivity and specificity of 82% and 86% (AUC=90) while the peak systolic tissue velocity of 12 cm/s gave a sensitivity of 83% and a specificity of 71% (AUC=79).

Peak systolic 2D strain is deteriorated in ARVC patients and may help to characterize arrythmogenic right ventricular cardiomyopathy.