Coronary Fistulas in a Patient with a Novel Long QT Syndrome Mutation

Introduction

The congenital long QT syndrome (LQTS) is a genetic cardiac channelophathy with variable penetrance characterized by corrected QT (QTc) interval prolongation and predisposition to polymorphic ventricular tachycardia (PVT), which normally presents with syncope or sudden death. It can be caused by mutations in different ion channels, resulting in prolongation of the myocardial repolarization. The clinical course of LQTS throughout a patient’s lifetime is significantly influenced by the genotype and acquired factors that additionally compromise myocardial repolarization, including drug use, serum electrolyte abnormalities, autoimmune diseases, severe bradycardia, acute heart failure decompensation and myocardial ischemia.

This report presents the first description of PVT precipitated by myocardial ischemia secondary to multiple coronary fistulas in a patient with a novel LQTS-causing mutation.

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