Please confirm that an ethics committee approval has been applied for or granted: Not relevant (see information at the bottom of this page)

Background and Aims Malignant hyperthermia (MH) is a rare, complex, and life-threatening genetic disorder of skeletal muscle characterized by a hypermetabolic response to triggering agents. MH susceptibility is often associated with mutations in the RYR1 and CACNA1S genes and affects approximately 1:2 000 to 1:3 000 individuals. Prompt recognition and treatment are crucial to prevent severe morbidity and mortality.

Methods Female, 23 years, with heterozygosity for the RYR1 variant, a known risk factor for MH, ASA I. Proposed for elective osteotomy of the trapezius-metacarpal joint of the right hand. Her mother had previously experienced a perioperative cardio-respiratory arrest, also carrying the same genetic variant. The patient was scheduled for the first surgical time and the operating room and team were prepared according to the institutional protocol for MH. Intraoperative monitoring included ASA standard and peripheral temperature. Ultrasound-guided block of right brachial plexus block (axillar approach) and intercostobrachial nerve with 30 mL ropivacaine 0.5% were performed. Moderate sedation was achieved with propofol. Intravenous analgesia was also administered intraoperatively. She remained hemodynamically stable throughout the procedure and in the postoperative period. The patient was discharged from hospital the day after the surgery, without complications.

Conclusions This case highlights the successful management of a patient with known MH susceptibility during elective surgery. Careful planning, avoidance of MH-triggering agents, and a prepared multidisciplinary team were critical to the patient‘s safety. Vigilance, communication, and adherence to MH guidelines remain critical to minimize the risks associated with this rare but potentially life-threatening condition.