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

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

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...

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Main Authors: Frank Schuster, Carsten Wessig, Christoph Schimmer, Stephan Johannsen, Marc Lazarus, Ivan Aleksic, Rainer Leyh, Norbert Roewer
Format: Article
Language:English
Published: Hindawi Limited 2012-01-01
Series:Case Reports in Anesthesiology
Online Access:http://dx.doi.org/10.1155/2012/349046
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spelling doaj-84a86f91d0e44a3fbd170521897dfe182020-11-25T01:06:28ZengHindawi LimitedCase Reports in Anesthesiology2090-63822090-63902012-01-01201210.1155/2012/349046349046In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac TransplantationFrank Schuster0Carsten Wessig1Christoph Schimmer2Stephan Johannsen3Marc Lazarus4Ivan Aleksic5Rainer Leyh6Norbert Roewer7Department of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Neurology, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Cardiothoracic and Thoracic Vascular Surgery, University of Wuerzburg, 97080 Wuerzburg, GermanyDepartment of Anaesthesia and Critical Care, University of Wuerzburg, 97080 Wuerzburg, GermanyEmery-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.http://dx.doi.org/10.1155/2012/349046
collection DOAJ
language English
format Article
sources DOAJ
author Frank Schuster
Carsten Wessig
Christoph Schimmer
Stephan Johannsen
Marc Lazarus
Ivan Aleksic
Rainer Leyh
Norbert Roewer
spellingShingle Frank Schuster
Carsten Wessig
Christoph Schimmer
Stephan Johannsen
Marc Lazarus
Ivan Aleksic
Rainer Leyh
Norbert Roewer
In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
Case Reports in Anesthesiology
author_facet Frank Schuster
Carsten Wessig
Christoph Schimmer
Stephan Johannsen
Marc Lazarus
Ivan Aleksic
Rainer Leyh
Norbert Roewer
author_sort Frank Schuster
title In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
title_short In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
title_full In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
title_fullStr In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
title_full_unstemmed In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation
title_sort in vitro contracture test results and anaesthetic management of a patient with emery-dreifuss muscular dystrophy for cardiac transplantation
publisher Hindawi Limited
series Case Reports in Anesthesiology
issn 2090-6382
2090-6390
publishDate 2012-01-01
description 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.
url http://dx.doi.org/10.1155/2012/349046
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AT carstenwessig invitrocontracturetestresultsandanaestheticmanagementofapatientwithemerydreifussmusculardystrophyforcardiactransplantation
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