Document Detail

Successful surgical repair for Emery-Dreifuss muscular dystrophy valvular disease with long-term follow-up.
MedLine Citation:
PMID:  20139205     Owner:  NLM     Status:  MEDLINE    
Emery-Dreifuss muscular dystrophy (EDMD) is an X-linked muscular dystrophy in which cardiac involvement can be serious. The disease progresses rapidly and the prognosis is strongly associated with cardiac involvement. We present the case of a 49-year-old man who was admitted with shortness of breath. Echocardiography revealed a huge right atrium and severe tricuspid regurgitation caused by annular dilatation and destruction of leaflets and chordae by pacemaker leads. Tricuspid valve replacement, right atrial plication and implantation of epicardial pacemaker leads were performed. The patient is in a good condition seven years after surgery. Cardiac transplantation is the desired therapy for valvular disease in EDMD cardiomyopathy, but this case indicates that valve replacement is also acceptable if performed with the appropriate timing.
Hideaki Takai; Takafumi Yamada; Seiichi Tada; Ichiro Matsumaru
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-02-05
Journal Detail:
Title:  Interactive cardiovascular and thoracic surgery     Volume:  10     ISSN:  1569-9285     ISO Abbreviation:  Interact Cardiovasc Thorac Surg     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-04-20     Completed Date:  2010-08-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101158399     Medline TA:  Interact Cardiovasc Thorac Surg     Country:  England    
Other Details:
Languages:  eng     Pagination:  811-2     Citation Subset:  IM    
Copyright Information:
2010 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
Department of Cardiovascular Surgery, Oita Prefectural Hospital, Oita-city, Oita Prefecture, Japan.
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MeSH Terms
Cardiomyopathies / etiology,  physiopathology,  therapy
Cardiopulmonary Bypass / methods
Combined Modality Therapy
Echocardiography, Doppler
Follow-Up Studies
Heart Atria / pathology,  surgery
Heart Valve Prosthesis*
Heart Valve Prosthesis Implantation / methods*
Middle Aged
Muscular Dystrophy, Emery-Dreifuss / complications*,  diagnosis
Pacemaker, Artificial
Preoperative Care / methods
Risk Assessment
Severity of Illness Index
Tomography, X-Ray Computed / methods
Treatment Outcome
Tricuspid Valve Insufficiency / diagnosis,  etiology*,  surgery*

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