Objective This study was designed to review MRI characteristics and assessed its risk factors for life-threatening ventricular arrhythmia in arrhythmogenic right ventricular cardiomyopathy (ARVC).
Methods We collected a consecutive series of 63 patients with clinical diagnosis of ARVC at a single institution. In all cases the diagnosis was performed according to ESC/ISFC diagnostic criteria. MRI characteristics were compared between patients with syncope and concomitantly sustained ventricular tachycardia or ventricular fibrillation (group 1) and remaining patients (group 2). Morphological and functional parameters and tissue differentiation were assessed.
Results Univariate analysis showed significantly differences between both groups in terms of familial history of ARVC or sudden death (14% vs 41%, p=0.015), the accordion sign (58% vs 81%, p=0.031), left ventricular (LV) involvement (47% vs 74%, p=0.032), number of regions with intramyocardial fat infiltration (2.4±1.4 vs 3.1±1.5, p=0.047), number of regions with myocardial fibrosis (1.0±0.9 vs 1.6±0.9, p=0.013). No differences were noted when comparing baseline characteristics of the patient population. A binary logistic regression model showed that familial history of ARVC or sudden death (OR=7.300, 95% CI 1.606 to 33.177, p=0.010), the accordion sign (OR=7.000, 95% CI 1.509 to 32.468, p=0.013) and number of regions with myocardial fibrosis (OR=2.204, 95% CI 1.116 to 4.354, p=0.023) were independent predictors for life-threatening ventricular arrhythmia in ARVC.
Conclusions MRI is the optimal imaging approach for detecting ARVC. Familial history of ARVC or sudden death, the accordion sign and number of regions with myocardial fibrosis were associated with an increased risk of life-threatening ventricular arrhythmia in patients with ARVC.