%0 Journal Article
%T In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
%A Frank Schuster
%A Carsten Wessig
%A Christoph Schimmer
%A Stephan Johannsen
%A Marc Lazarus
%A Ivan Aleksic
%A Rainer Leyh
%A Norbert Roewer
%J Case Reports in Anesthesiology
%D 2012
%I Hindawi Publishing Corporation
%R 10.1155/2012/349046
%X 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.
%U http://www.hindawi.com/crim/anesthesiology/2012/349046/