Late Gadolinium Enhancement at cardiac magnetic resonance predicts malignant ventricular arrhythmias in systemic sclerosis

Abstract Background Malignant arrhythmias due to cardiac involvement are a frequent cause of death in systemic sclerosis (SSc). Cardiac involvement, which is linked to both ischaemic and non-ischaemic fibrotic deposition, is often subclinical. Cardiovascular magnetic resonance (CMR) is the non-invasive gold standard for myocardial tissue characterization and can detect macroscopic myocardial fibrosis through late Gadolinium enhancement (LGE). Aim To evaluate the role of LGE to predict malignant arrhythmias requiring implantable cardioverter defibrillator (ICD) in SSc. Methods 289 SSc patients underwent a thorough clinical evaluation and CMR exam using a 1.5 T scanner. Biventricular function parameter by SSFP cine images, oedema by STIR T2 images, and macroscopic fibrosis by LGE were assessed. Patients were followed-up and malignant ventricular arrhythmias requiring ICD implantation was considered as event. Results Out of 289 patients, 111 (38.4%) showed LGE and 83/111 (28.7% of the total population) showed a non-junctional distribution. During the follow-up (45±27 months), 10 patients needed ICD after malignant ventricular arrhythmias (7 patients with LGE, 3 patients without LGE). CMR predictors of cardiac events by univariate analysis were left and right ventricular ejection fractions, indexed right atrial area and non-junctional LGE. At multivariate analysis, macroscopic myocardial fibrosis detected by LGE was an independent predictor (hazard ratio 5.4; 95% C.I. 1.1–28.8, p