Remimazolam as an Adjunct to General Anesthesia During Surgery for Congenital Heart Disease in a Pediatric Patient With a Family History of Malignant Hyperthermia.

Malignant hyperthermia (MH) is an inherited disorder of muscle physiology, triggered by various pharmacologic agents including succinylcholine or the volatile anesthetic agents. In susceptible patients, intraoperative care may be provided by total intravenous anesthesia (TIVA) with propofol or other sedative-hypnotic agents. Remimazolam is a novel benzodiazepine which possesses sedative, anxiolytic, and amnestic properties similar to those of midazolam. Metabolism by tissue esterases results in a half-life of 5 - 10 min, a limited context sensitive half-life, which may make it a suitable agent for continuous administration during TIVA. We present anecdotal experience with the use of remimazolam as an adjunct to general anesthesia during the intraoperative care of a 6-month-old patient undergoing surgery for congenital heart disease with cardiopulmonary bypass. Previous reports regarding the use of remimazolam in MH-susceptible patients are reviewed, and its role in this clinical scenario is discussed.