Total intravenous anesthesia for cardiac transplantation in a teenager with Becker muscular dystrophy: A case report

Becker muscular dystrophy (BMD) is a genetic disorder caused by partial deficiency of dystrophin, leading to progressive skeletal muscle weakness and cardiac failure. Up to one-third of affected individuals develop dilated cardiomyopathy, eventually requiring heart transplantation at a young age. Although BMD is not directly linked to malignant hyperthermia (MH), patients may experience severe MH-like reactions, particularly when exposed to succinylcholine or volatile anesthetics. Therefore, total intravenous anesthesia (TIVA) is often preferred in this population, despite the cardioprotective benefits of volatile agents through myocardial preconditioning. We report the case of a teenager with BMD and advanced cardiomyopathy who successfully underwent heart transplantation under TIVA. Anesthesia was maintained with target-controlled infusion (TCI) of propofol and remifentanil. Additional boluses of ketamine and midazolam were administered to maintain a bispectral index (BIS) between 40 and 60. Postoperatively, the patient was admitted to the intensive care unit receiving low-dose infusions of dobutamine, sodium nitroprusside, and inhaled nitric oxide, and was safely extubated 3 h later. This case highlights the feasibility and safety of TIVA in patients with BMD undergoing major cardiac surgery.