0297: Risk stratification using CMR delayed enhancement in LMNA mutation carriers

Abstract

Laminopathy is a frequent cause of familial dilated cardiomyopathy (DCM) associated with peripheral myopathy. The clinical cardiac manifestations are usually atrial fibrillation, atrio-ventricular blocks with a DCM. Myocardial delayed enhancement (DE) with cardiac magnetic resonance imaging (CMR) is known to be associated with a worse rhythmic prognosis in DCM. Determine if myocardial DE can be used as a risk stratification tool for cardiac prognosis in asymptomatic LMNA mutation carriers. Cohort study of 15 LMNA mutation carriers with left ventricular ejection fraction (LVEF) > 50% followed between 2009 et 2013 (10 DE+, 5 DE-). Primary endpoint was rythmic events uprise and secondary endpoint was LVEF evolution. Regarding patients with DE since 2009, 60% required pacemaker or implantable cardioverter-defibrillator (ICD) implantation for atrio-ventricular blocks, ventricular arrhythmias or left ventricular dysfunction whereas no patient with a normal CMR did (p = 0,027). In 2009, LVEF was similar in the two groups (LVEF = 68% DE+ group vs 65% DE- group, p = 0,18), whereas in 2013, DE group has a lower LVEF (LVEF = 53% DE+ group vs 65% DE- group, p = 0,009). Myocardial DE seems to be an early cardiac marker for PM or ICD implantation risk and left ventricular dysfunction in LMNA mutation carriers with normal LVEF.