UC Davis

BACKGROUND Long QT syndrome (LQTS) is an arrhythmogenic heart condition that can be congenital or acquired. Prolonged ventricular repolarizations in individuals with the disorder can cause fatal arrhythmias. Abnormal functioning of cardiac ion channels leads to arrhythmias such as torsades de pointes (TdP) and may be triggered by stress or medications. Many medications used in the perioperative period are triggers for the arrythmia. CASE REPORT A 7-year-old patient with known congenital LQTS type 2 presented for bilateral myringotomy and tube placement. The patient was otherwise healthy and taking propranolol daily. Preoperative midazolam was administered for anxiolysis, and induction of anesthesia was uneventful. He sustained an episode of TdP immediately following general anesthetic induction after failure of an in situ automatic implantable cardioverter-defibrillator (AICD). External defibrillation succeeded, and the patient was stabilized in the Postanesthesia Recovery Unit before transfer to the Pediatric Intensive Care Unit. Interrogation of the AICD revealed several undelivered defibrillation attempts. A chest X-ray showed an area suggestive of an epicardial electrode fracture. The following day, the AICD was replaced with no arrythmias noted. The patient had an uneventful recovery. CONCLUSIONS In patients with a known history of LQTS, preparation and prevention are cornerstones of anesthesia care. Minimizing the use of triggering medications and emotional stress in the perioperative period, combined with ready equipment and medications to respond to arrythmias, are essential. In children, there is a greater chance of lead fracture and resulting device failure. Preoperative history of device function or interrogation of the AICD and possibly a chest X-ray are essential to ensure the integrity of the leads.