Document Detail


Delayed contrast enhancement on cardiac MRI unmasks subclinical cardiomyopathy in a case of myotonic dystrophy type 2.
MedLine Citation:
PMID:  22796821     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Current evidence suggests cardiac involvement and electrocardiographic changes of increasing frequency with age in patients with myotonic dystrophy type 2 (DM2). Myocyte hypertrophy with concurrent fibrosis seems to be the anatomical correlate. Moreover, morphological and functional changes indicative of subclinical cardiomyopathy have been demonstrated by means of cardiac magnetic resonance imaging (CMRI) and spectroscopy in patients with no overt cardiac disease. We present a 68-year-old woman with genetically established DM2 and no clinical, electrocardiographic or echocardiographic signs indicative of cardiac involvement. CMRI revealed delayed contrast enhancement of the anterior portion of the interventricular septum, indicating myocardial involvement. Contrast-enhanced CMRI might be a useful diagnostic tool in assessing cardiac involvement in cases of DM2. The role of delayed contrast enhancement should be further investigated in order to elucidate the cardiac features of this fascinating multisystem disease.
Authors:
Konstantinos Spengos; Elias Gialafos; Sofia Vassilopoulou; Panagiotis Toulas; Panagiota Manta
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Hellenic journal of cardiology : HJC = Hellēnikē kardiologikē epitheōrēsē     Volume:  53     ISSN:  2241-5955     ISO Abbreviation:  Hellenic J Cardiol     Publication Date:    2012 Jul-Aug
Date Detail:
Created Date:  2012-07-16     Completed Date:  2012-12-11     Revised Date:  2013-08-07    
Medline Journal Info:
Nlm Unique ID:  101257381     Medline TA:  Hellenic J Cardiol     Country:  Greece    
Other Details:
Languages:  eng     Pagination:  324-6     Citation Subset:  IM    
Affiliation:
University of Athens Medical School, Department of Neurology, Eginition Hospital, Athens, Greece. kspengos@otenet.gr
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MeSH Terms
Descriptor/Qualifier:
Aged
Cardiomyopathies / diagnosis*,  etiology
Echocardiography
Electrocardiography
Electromyography
Female
Humans
Magnetic Resonance Imaging / methods*
Myotonic Disorders / complications*

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