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

Document Type : Case Report


Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.


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

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