{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T14:55:25Z","timestamp":1793199325589,"version":"4.5.0"},"reference-count":35,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2011,3,1]],"date-time":"2011-03-01T00:00:00Z","timestamp":1298937600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>In vitro studies have shown impairment of energy metabolism in cardiac fibroblasts from Fabry patients. A recent in vivo study reported an association between cardiac energy metabolism and increased myocardial mass in Fabry patients. We therefore assessed possible disturbances of cardiac energy metabolism in Fabry patients by in vivo31P-MR-spectroscopy. Additionally, the effect of enzyme replacement therapy (ERT) on cardiac energetics was tested.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>Twenty-three patients (41 \u00b1 9 years; 10 females) with genetically proven Fabry disease were examined with a 1.5 T Scanner, and compared with an age-matched healthy control group. Eight patients underwent ERT and had follow-up examinations after 3 and 14 months. The high-energy phosphate molecules phosphocreatine (PCr) and adenosine triphosphate (ATP) were quantified in localized 31P-spectra by SLOOP (spectral localization with optimum point spread function). Cine- and late gadolinium enhancement (LGE) studies were also performed. When compared with healthy controls, Fabry patients demonstrated reduced PCr- (6.1 \u00b1 1.9 vs. 8.8 \u00b1 2.6 mmol\/kg; P = 0.003) and ATP concentrations (3.9 \u00b1 1.5 vs. 4.6 \u00b1 1.0 mmol\/kg; P = 0.048). During ERT, PCr concentrations increased (7.1 \u00b1 1.5 mmol\/kg vs. 6.1 \u00b1 1.9; P &amp;lt; 0.05) and left ventricular mass decreased (215 \u00b1 55 vs. 185 \u00b1 45 g; P = 0.012). Disturbances in cardiac energetics were not correlated to the presence or absence of cardiac fibrosis on LGE.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Cardiac energy metabolism is disturbed in Fabry disease; this may play an important role in the pathogenesis of Fabry cardiomyopathy. Enzyme replacement therapy ameliorates energetic depression.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/eurjhf\/hfq211","type":"journal-article","created":{"date-parts":[[2010,12,13]],"date-time":"2010-12-13T23:09:17Z","timestamp":1292281757000},"page":"278-283","source":"Crossref","is-referenced-by-count":45,"title":["Cardiac Energy Metabolism is Disturbed in Fabry Disease and Improves with Enzyme Replacement Therapy Using Recombinant Human Galactosidase A"],"prefix":"10.1093","volume":"13","author":[{"given":"Wolfram","family":"Machann","sequence":"first","affiliation":[{"name":"Institut f\u00fcr R\u00f6ntgendiagnostik, Universit\u00e4t W\u00fcrzburg , Josef-Schneider-Str. 2, 97080, W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Breunig","sequence":"additional","affiliation":[{"name":"Medizinische Klinik und Poliklinik 1, Universit\u00e4t W\u00fcrzburg , W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Weidemann","sequence":"additional","affiliation":[{"name":"Medizinische Klinik und Poliklinik 1, Universit\u00e4t W\u00fcrzburg , W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rn","family":"Sandstede","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr R\u00f6ntgendiagnostik, Universit\u00e4t W\u00fcrzburg , Josef-Schneider-Str. 2, 97080, W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dietbert","family":"Hahn","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr R\u00f6ntgendiagnostik, Universit\u00e4t W\u00fcrzburg , Josef-Schneider-Str. 2, 97080, W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herbert","family":"K\u00f6stler","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr R\u00f6ntgendiagnostik, Universit\u00e4t W\u00fcrzburg , Josef-Schneider-Str. 2, 97080, W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Neubauer","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, John Radcliffe Hospital, Oxford University , Oxford ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Wanner","sequence":"additional","affiliation":[{"name":"Medizinische Klinik und Poliklinik 1, Universit\u00e4t W\u00fcrzburg , W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meinrad","family":"Beer","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr R\u00f6ntgendiagnostik, Universit\u00e4t W\u00fcrzburg , Josef-Schneider-Str. 2, 97080, W\u00fcrzburg ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,2,18]]},"reference":[{"issue":"Suppl. 2","key":"2026020203310585200_ejhfhfq211-bib-0001","first-page":"S172","article-title":"Fabry disease: clinical spectrum and evidence-based enzyme replacement therapy","volume":"2","author":"Desnick","year":"2006","journal-title":"Nephrol Ther"},{"key":"2026020203310585200_ejhfhfq211-bib-0002","doi-asserted-by":"crossref","first-page":"S181","DOI":"10.1046\/j.1523-1755.63.s84.5.x","article-title":"Fabry disease: diagnosis and treatment","volume":"63","author":"Breunig","year":"2003","journal-title":"Kidney Int Suppl"},{"key":"2026020203310585200_ejhfhfq211-bib-0003","doi-asserted-by":"crossref","first-page":"1515","DOI":"10.1016\/j.amjcard.2005.11.087","article-title":"Impact of enzyme replacement therapy on cardiac morphology and function and late enhancement in Fabry's cardiomyopathy","volume":"97","author":"Beer","year":"2006","journal-title":"Am J Cardiol"},{"key":"2026020203310585200_ejhfhfq211-bib-0004","doi-asserted-by":"crossref","first-page":"2151","DOI":"10.1016\/j.ehj.2003.09.017","article-title":"Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. 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