| Successful surgical repair for Emery-Dreifuss muscular dystrophy valvular disease with long-term follow-up. | |
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MedLine Citation:
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PMID: 20139205 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Hideaki Takai; Takafumi Yamada; Seiichi Tada; Ichiro Matsumaru |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-02-05 |
Journal Detail:
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Title: Interactive cardiovascular and thoracic surgery Volume: 10 ISSN: 1569-9285 ISO Abbreviation: Interact Cardiovasc Thorac Surg Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-04-20 Completed Date: 2010-08-11 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101158399 Medline TA: Interact Cardiovasc Thorac Surg Country: England |
Other Details:
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Languages: eng Pagination: 811-2 Citation Subset: IM |
Copyright Information:
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2010 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. |
Affiliation:
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Department of Cardiovascular Surgery, Oita Prefectural Hospital, Oita-city, Oita Prefecture, Japan. cvstakai@yahoo.co.jp |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cardiomyopathies
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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* Humans Male Middle Aged Muscular Dystrophy, Emery-Dreifuss / complications*, diagnosis Pacemaker, Artificial Preoperative Care / methods Risk Assessment Severity of Illness Index Time Tomography, X-Ray Computed / methods Treatment Outcome Tricuspid Valve Insufficiency / diagnosis, etiology*, surgery* |
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