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Case Reports in Anesthesiology
Volume 2012, Article ID 349046, 4 pages
http://dx.doi.org/10.1155/2012/349046
Case Report

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

1Department of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, Germany
2Department of Neurology, University of Wuerzburg, 97080 Wuerzburg, Germany
3Department of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, Germany

Received 3 June 2012; Accepted 31 July 2012

Academic Editors: S. Faenza, R. S. Gomez, A. Han, and E. A. Vandermeersch

Copyright © 2012 Frank Schuster et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.