Cardiomyopathy in Duchenne Muscular Dystrophy: Current Value of Clinical, Electrophysiological and Imaging Findings in Children and Teenagers.
Klin Padiatr
; 227(4): 225-31, 2015 Jul.
Article
em En
| MEDLINE
| ID: mdl-26058601
ABSTRACT
BACKGROUND:
Progressive cardiomyopathy (CMP) is one main cause of death in DMD. This cross-sectional assessment of different cardiac diagnostic procedures focusses on preterm diagnosis of cardiac dysfunction. PATIENTS 39 male DMD patients aged 6-20 years were included. 6 patients were still ambulatory, 21 patients received corticosteroid therapy.METHODS:
All patients were investigated by ECG, Holter ECG and heart rate variability (HRV), B-type natriuretic peptide (BNP), echocardiography (TTE), tissue Doppler Imaging (TD) and magnetic resonance imaging (MRI) with Late Gadolinium enhancement (LE) and segmental wall motion analysis (WMA).RESULTS:
56% of the patients showed repolarization abnormalities and 76% altered HRV. Subnormal ventricular function was found in 25% by TTE and in 34% by MRI. TD differed from normal controls only in the apical septum. In MRI 89% of the patients showed different distribution and intensity of LE and WM restriction. The extent of LE was less in patients after steroid treatment (p<0.05).DISCUSSION:
MRI with segmental LE- and WM-analysis seems to be superior to TTE and TD in exploring regional distribution and severity of damage of the myocardium. ECG and HRV abnormalities are common in DMD-patients but not tightly predictive for segmental and global left ventricular dysfunction. Targeted treatment of CMP in DMD needs prospective evaluation.CONCLUSION:
A timely cardiac MRI is the most sensitive investigation for the identification of early myocardial changes in DMD which is a prerequisite for early interventions and therapeutic strategies.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Diagnóstico por Imagem
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Eletrocardiografia Ambulatorial
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Peptídeo Natriurético Encefálico
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Distrofia Muscular de Duchenne
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Eletrocardiografia
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Frequência Cardíaca
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Contração Miocárdica
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Cardiomiopatias
Tipo de estudo:
Diagnostic_studies
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Prognostic_studies
Limite:
Adolescent
/
Adult
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Child
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Humans
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Male
Idioma:
En
Revista:
Klin Padiatr
Ano de publicação:
2015
Tipo de documento:
Article
País de afiliação:
Alemanha