Anaesthetic considerations for a patient with Emery-Dreifuss muscular dystrophy undergoing cardiac resynchronisation...

Emery-Dreifuss muscular dystrophy is a rare inherited neuromuscular disorder characterised by early joint contractures, slowly progressive humero-peroneal weakness and cardiac conduction defects or cardiomyopathy. Although contractures and weakness usually begin in childhood, cardiac complications, such as atrioventricular block, arrhythmias and dilated cardiomyopathy, typically emerge in early adulthood and may cause sudden cardiac death, if unrecognised. Anaesthetic management is challenging due to potential airway complications from cervical contractures, restrictive respiratory physiology and cardiac instability. Pre-operative cardiac and pulmonary assessment is essential. Depolarising neuromuscular blocking agents and volatile anaesthetics are not absolutely contraindicated but are preferably avoided due to the risk of rhabdomyolysis or malignant hyperthermia-like reactions. Total intravenous anaesthesia is preferred to minimise these risks. We report a 27-year-old man with genetically confirmed Emery-Dreifuss muscular dystrophy and severe multisystem involvement who underwent cardiac resynchronisation therapy pacemaker implantation under total intravenous anaesthetic technique with rocuronium and reversal with sugammadex. Anaesthetic management focused on malignant hyperthermia precautions, airway preparation for limited cervical mobility and minimising arrhythmia risk with readiness for external cardiac pacing. The procedure and recovery were uneventful, demonstrating that the total intravenous anaesthetic technique can be an effective technique for patients with Emery-Dreifuss muscular dystrophy undergoing device implantation.