Combined inhibition of Na+ and Ca2+ channels: A novel paradigm for the treatment of incessant ventricular arrhythmias in Andersen-Tawil syndrome

Abstract

Introduction Andersen-Tawil syndrome type 1 (ATS1), also referred to as long QT syndrome type 7, is a disorder of ventricular repolarization caused by mutations in the KCNJ2 gene. The cardiac phenotype is characterized by bidirectional ventricular tachycardia (VT), polymorphic VT, and multifocal premature ventricular contractions, with or without corrected QT prolongation. In addition, patients manifest facial dysmorphisms and periodic skeletal muscle paralysis. Treatment of ventricular arrhythmia in ATS1 can be difficult, and patients with a high burden of ventricular ectopy can develop tachycardia-induced cardiomyopathy. In this case report, we describe a novel approach to the treatment of refractory ventricular arrhythmia in an adolescent patient with ATS1.