The atrial phenotype of the inherited primary arrhythmia syndromes

Abstract

Over the past two decades, our understanding of inherited primary arrhythmia syndromes has been enriched by studies that have aimed to define the clinical characteristics and the genetic, cellular and molecular features predisposing patients to an enhanced risk of ventricular arrhythmias. In contrast, very little is known about the causative role of inherited cardiac channelopathies on atrial conduction abnormalities possibly leading to different atrial tachyarrhythmias. The diagnostic and therapeutic management of patients with an inherited cardiac channelopathy presenting with atrial arrhythmias remains highly challenging and is in urgent need of improvement. This review will assess the current knowledge on atrial electrical abnormalities affecting patients with different forms of inherited primary arrhythmia syndromes, including long and short QT syndromes, early repolarisation syndrome, catecholaminergic polymorphic ventricular tachycardia and Brugada syndrome.