Processed Electroencephalography-Guided Total Intravenous Anesthesia in a Malignant Hyperthermia-Susceptible Child Undergoing Ventricular Septal Defect Repair: A Case Report

Malignant hyperthermia (MH) is a rare, life-threatening reaction to certain anesthetic medications that can occur during surgery.Managing an MH-susceptible child for congenital cardiac surgery is particularly challenging: cardiopulmonary bypass (CPB), with its attendant hypothermia and subsequent rewarming, can obscure the early signs of an MH crisis.Total intravenous anesthesia (TIVA) is utilized for MH-susceptible patients, but titrating anesthetic depth during CPB can be complicated, and processed electroencephalography (pEEG) has been studied sparingly in the pediatric cardiac population.We describe the anesthetic management of a two-year-old, 10.4-kg child with a likely pathogenic variant in the ryanodine receptor 1 (RYR1) gene who underwent ventricular septal defect (VSD) repair with CPB.A trigger-free environment was established, general anesthesia was maintained with a propofol-based TIVA technique, and anesthetic depth was monitored with a SedLine® brain function monitor (Masimo Corporation, Irvine, USA), using the raw electroencephalogram (EEG), spectral edge frequency 95 (SEF95), and density spectral array (DSA).The intraoperative course was uneventful, and no features of MH developed.This case illustrates the feasibility of pEEG-guided TIVA in an MH-susceptible child undergoing congenital cardiac surgery with CPB, and the value of interpreting the spectral display rather than a single index value during hypothermia and rewarming.