Undiagnosed long QT syndrome turns fatal in a child scheduled for cochlear implant

Sir, Long QT syndrome (LQTS) is a cardiac repolarization disorder. Catecholamine surge or several drugs can prolong QT interval and precipitate torsade de pointes (TdP) and cardiac arrest.[1] A 2.5-year-old male child weighing 12 kg was scheduled for a cochlear implant for sensorineural hearing loss (SNHL). He had global developmental delay and recurrent history of breath-holding spells (BHSs) since 10 months of age which were precipitated by excessive crying. No treatment was advised by the pediatrician for the BHS. This episode terminated with a painful stimulus. Family history and other systemic examinations were normal. In the operating room, the American Society of Anesthesiologists standard monitors were attached, and general anesthesia was induced according to institutional protocol. Surgery was uneventful lasting 360 min, and the patient was shifted to the postanesthesia care unit (PACU) after extubation. After 30 min of PACU stay, the child suddenly became unresponsive. In the multipara patient monitor, an electrocardiogram (ECG) showed TdP-like picture. Cardiopulmonary resuscitation was initiated, and an injection of magnesium sulfate (30 mg/kg) was given intravenously. Ten minutes later, return of spontaneous circulation was achieved. Postresuscitation ECG showed a QTc interval of 508 ms [Figure 1]. A cardiologist’s consultation was sought, and he suggested a possibility of congenital LQTS or Jervell and Lange-Nielson syndrome (JLNS). Injection esmolol infusion (100 μg/kg/min) was started and shifted to the pediatric intensive care unit. However, the child continued to deteriorate and developed refractory cardiogenic shock and succumbed to it on postoperative day 2.Figure 1: Postresuscitation electrocardiogram showing QTc interval 508 msJLNS is the most severe variant of LQTS. It is characterized by congenital profound bilateral SNHL and long QTc >500 ms.[2] Syncope has also been reported during emotional stress or exercise. However, only 60% of patients are symptomatic at the time of diagnosis, and around 25%–40% of patients have a nondiagnostic resting QTc (“concealed LQTS”).[3,4] In our patient, history of syncope got misdiagnosed as BHS, and no active treatment was advised. If he was diagnosed as JLNS preoperatively, we could have avoided drugs such as inhalational agents, glycopyrrolate, and ondansetron during surgery as these drugs can prolong QTc interval. Successful anesthetic management of patients with LQTS requires adequate anxiolysis, smooth induction, and extubation, and keeping patients’ pain-free, as any kind of sympathetic stimulation can precipitate malignant arrhythmia. All the drugs causing QT prolongation should be listed and avoided.[1] In conclusion, LQTS must be suspected in pediatric patients with a syncope history. Drugs increasing QT interval should be avoided in the perioperative period and propofol infusion for total intravenous anesthesia might be thought of as a safe option for maintaining anesthesia in individuals who are suspected of having it. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.