0211 : Implications of baselines 2010 task force criterias on ventricular arrhythmias in ARVC

International audienceIntroduction Arrythmogenic right ventricular cardiomyopathy (ARVC) is an autosomic dominant disease with a variable penetrance. Based on 2010 task force criteria, baselines characteristics of patients referred from 3 French centers were analyzed to identified risk factors for ventricular arrhythmias. Methods All patients with a diagnosis of ARVC referred from 3 academic French centers between 2006 and 2014 were included. The diagnosis was based on the 2010 Task Force. Occurrence of sustained ventricular arrhythmias and date of last alive status were collected from each center. Results The population consisted in 259 patients (171 males, sex ratio 1.9) with a mean age at diagnosis of 40+/-17 years. The VA group is composed of 61 patients (23.5%) who experienced at least 1.9 VT episodes. An ICD was implanted in 59% of them. Mean follow-up was 4.0 years in “no VA group” and 7.5 years in “VA group” (p=0.0003). Occurrence of syncope (OR=2.26, 95%CI [0.99-5.0], p=0.03) or VA (OR=3.1, 95%CI[1.8-5.2], p<0.0001) and sustained or non-sustained VA during exercise testing were associated (OR=4.2, 95%CI [0.90-19.5], p=0.03) with VA during follow-up. We failed to identify any ECG parameter related to the occurrence of VA. Dilated right ventricular (RV) was significantly associated with VA during follow-up (OR = 3.6, 95%CI [1.36;9.65], p=0.005). Severe RV dysfunction was more often identified (10% vs 3%, p=0.06) in the “VA group” in echocardiography. By MRI, RVEF was significantly lower in “VA group” (34.6% vs 42.1%, p=0.03) but RV end diastolic volume and presence of akinesia or dyskinesia were not significantly different between two groups. Genetic screening is on-going for all patients. Genetic screening will be performed using HaloPlex™ Target Enrichment System (Agilent Technologies) which allow the sequencing of 163 genes previously reported as involved in cardiac arrhythmias, conduction defect and cardiomyopathies. Conclusion Occurrence of syncope and VA at baseline or during exercise test are associated with occurrence of VA during follow-up. RV abnormalities on echocardiography and MRI are significantly associated with VT