INTERATRIAL BLOCK AS A FIRST CLINICAL PRESENTATION OF ATRIAL CARDIOMYOPATHY RELATED TO A NOVEL LMNA VARIANT: A CASE REPORT

Abstract

Abstract Interatrial block (IAB) is a conduction delay in Bachmann’s bundle with a well–described association with structural heart disease, supraventricular arrhythmias, and cardiovascular events. We report the case of an asymptomatic 35–year–old man in whom the presence of IAB at electrocardiogram led to a comprehensive evaluation including speckle–tracking echocardiography, 24 h Holter monitoring, and genetic testing. Speckle–tracking echocardiography demonstrated a decrease in the longitudinal strain of interventricular septum, a typical feature of LMNA–related cardiomyopathy, and decreased indices of left atrial deformation. A diagnosis of cardiac laminopathy related to the frame shift variant c.1367 (p.Asn456Thrfs*24) of the LMNA gene was made. A dual–chamber implantable cardioverter defibrillator implantation was performed for the high risk of life–threatening ventricular tachyarrhythmias. This case demonstrates that IAB could be a rare presentation of a life–threatening laminopathy. Strain echocardiography is an essential tool to evaluate the deposition of fibrosis tissue in subclinical cardiomyopathies. Our report describes a novel variant of LMNA gene associated with a high risk of sudden cardiac death.