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Myocardial lipid content in Fabry disease: a combined \(^1\)H-MR spectroscopy and MR imaging study at 3 Tesla
Zitieren Sie bitte immer diese URN: urn:nbn:de:bvb:20-opus-146693
- 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 \(^1\)H magnetic resonance spectroscopy (MRS) at 3 Tesla. Methods Myocardial lipid content was quantified in vivo by \(^1\)H-MRS in 30 patients (12 male, 18 female; 18 patients treated with enzyme replacementBackground 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 \(^1\)H magnetic resonance spectroscopy (MRS) at 3 Tesla. Methods Myocardial lipid content was quantified in vivo by \(^1\)H-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 \(^1\)H-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 \(^1\)H-MRS. Finally, the authors share the opinion that a comprehensive cardiac examination including three subsections (LGE; \(^1\)H-MRS; T\(_1\) mapping), could hold the highest potential for the final assessment of early and late myocardial changes in Fabry disease.…
Autor(en): | B. PetritschORCiD, H. Köstler, A. M. Weng, M. Horn, T. Gassenmaier, A. S. Kunz, F. Weidemann, C. Wanner, T. A. Bley, M. Beer |
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URN: | urn:nbn:de:bvb:20-opus-146693 |
Dokumentart: | Artikel / Aufsatz in einer Zeitschrift |
Institute der Universität: | Medizinische Fakultät / Institut für Medizinische Strahlenkunde und Zellforschung |
Medizinische Fakultät / Medizinische Klinik und Poliklinik I | |
Sprache der Veröffentlichung: | Englisch |
Titel des übergeordneten Werkes / der Zeitschrift (Englisch): | BMC Cardiovascular Disorders |
Erscheinungsjahr: | 2016 |
Band / Jahrgang: | 16 |
Heft / Ausgabe: | 205 |
Originalveröffentlichung / Quelle: | BMC Cardiovascular Disorders (2016) 16:205 DOI 10.1186/s12872-016-0382-4 |
DOI: | https://doi.org/10.1186/s12872-016-0382-4 |
Allgemeine fachliche Zuordnung (DDC-Klassifikation): | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 616 Krankheiten |
Freie Schlagwort(e): | Morbus Fabry; late gadolinium enhancement; lysosomal storage disease; magnetic resonance spectroscopy; myocardial lipid content; rare diseases |
Datum der Freischaltung: | 05.05.2017 |
Sammlungen: | Open-Access-Publikationsfonds / Förderzeitraum 2016 |
Lizenz (Deutsch): | CC BY: Creative-Commons-Lizenz: Namensnennung |