In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation

Abstract

Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by slowly progressive muscle weakness, early contractures, and dilated cardiomyopathy. We reported an uneventful general anaesthesia using total intravenous anaesthesia (TIVA) for cardiac transplantation in a 19-year-old woman suffering from EDMD. In vitro contracture test results of two pectoralis major muscle bundles of the patient suggest that exposition to triggering agents does not induce a pathological sarcoplasmic calcium release in the lamin A/C phenotype. However, due to the lack of evidence in the literature, we would recommend TIVA for patients with EDMD if general anaesthesia is required.

Anesthetic implications of muscular dystrophies

“…31 Aunque se ha reportado que aunque el test de contractura muscular in vitro es negativo para riesgo de hipertermia maligna, existen publicaciones que apoyan el uso de técnicas seguras como la anestesia total intravenosa. 32 Se han reportado dificultades para la intubación y para la anestesia regional epidural y espinal en estos pacientes.…”