Myocardial lipid content in Fabry disease: a combined 1H-MR spectroscopy and MR imaging study at 3 Tesla.
BMC Cardiovasc Disord
; 16(1): 205, 2016 10 28.
Article
em En
| MEDLINE
| ID: mdl-27793097
ABSTRACT
BACKGROUND:
Fabry disease is characterized by a progressive deposition of sphingolipids in different organ systems, whereby cardiac involvement leads to death. We hypothesize that lysosomal storage of sphingolipids in the heart as occurring in Fabry disease does not reflect in higher cardiac lipid concentrations detectable by 1H magnetic resonance spectroscopy (MRS) at 3 Tesla.METHODS:
Myocardial lipid content was quantified in vivo by 1H-MRS in 30 patients (12 male, 18 female; 18 patients treated with enzyme replacement therapy) with genetically proven Fabry disease and in 30 healthy controls. The study protocol combined 1H-MRS with cardiac cine imaging and LGE MRI in a single examination.RESULTS:
Myocardial lipid content was not significantly elevated in Fabry disease (p = 0.225). Left ventricular (LV) mass was significantly higher in patients suffering from Fabry disease compared to controls (p = 0.019). Comparison of patients without signs of myocardial fibrosis in MRI (LGE negative; n = 12) to patients with signs of fibrosis (LGE positive; n = 18) revealed similar myocardial lipid content in both groups (p > 0.05), while the latter showed a trend towards elevated LV mass (p = 0.076).CONCLUSIONS:
This study demonstrates the potential of lipid metabolic investigation embedded in a comprehensive examination of cardiac morphology and function in Fabry disease. There was no evidence that lysosomal storage of sphingolipids influences cardiac lipid content as measured by 1H-MRS. Finally, the authors share the opinion that a comprehensive cardiac examination including three subsections (LGE; 1H-MRS; T1 mapping), could hold the highest potential for the final assessment of early and late myocardial changes in Fabry disease.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Esfingolipídeos
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Espectroscopia de Ressonância Magnética
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Doença de Fabry
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Imagem Cinética por Ressonância Magnética
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Cardiomiopatias
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Miocárdio
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Guideline
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
BMC Cardiovasc Disord
Assunto da revista:
ANGIOLOGIA
/
CARDIOLOGIA
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Alemanha