Anesthetic Management of Jervell and Lange-Nielsen Syndrome With Long QT Undergoing Surgical Sympathectomy: A Pediatric Case Report

Abstract

<span>Jervell and Lange-Nielsen syndrome (JLNS) is an uncommon hereditary condition. Characterized by<span> deafness at birth, JLNS is associated with anomalies that distress the electrical system of the heart and<span> causes long QT syndrome. Patients with long QT syndrome are at risk of severe ventricular<span> arrhythmias. Every change in autonomic balance, particularly in the perioperative period, renders<span> patients prone to the risk for torsades de pointes and sudden cardiac arrest and death. Herein, we<span> describe a 6-year-old girl scheduled for left cardiac sympathetic denervation due to frequent<span> implantable cardioverter-defibrillator shocks.<br /></span></span></span></span></span></span></span>