In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
Keyword(s):
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.
2002 ◽
Vol 46
(9)
◽
pp. 1144-1149
◽
Keyword(s):
1981 ◽
Vol 39
◽
pp. 498-499
2012 ◽
Vol 302
(1)
◽
pp. C141-C153
◽
2008 ◽
Vol 649
(1-2)
◽
pp. 15-27
◽