{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:00:00Z","timestamp":1791216000979,"version":"4.1.0"},"reference-count":15,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2009,2,3]]},"abstract":"<jats:p>\n                    <jats:bold>\n                      <jats:italic>Background\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    Enzyme replacement therapy with recombinant \u03b1-galactosidase A reduces left ventricular hypertrophy and improves regional myocardial function in patients with Fabry disease during short-term treatment. Whether enzyme replacement therapy is effective in all stages of Fabry cardiomyopathy during long-term follow-up is unknown.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>\n                      <jats:italic>Methods and Results\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    We studied 32 Fabry patients over a period of 3 years regarding disease progression and clinical outcome under enzyme replacement therapy. Regional myocardial fibrosis was assessed by magnetic resonance imaging late-enhancement technique. Echocardiographic myocardial mass was calculated with the Devereux formula, and myocardial function was quantified by ultrasonic strain-rate imaging. In addition, exercise capacity was measured by bicycle stress test. All measurements were repeated at yearly intervals. At baseline, 9 patients demonstrated at least 2 fibrotic left ventricular segments (severe myocardial fibrosis), 11 had 1 left ventricular segment affected (mild fibrosis), and 12 were without fibrosis. In patients without fibrosis, enzyme replacement therapy resulted in a significant reduction in left ventricular mass (238\u00b142 g at baseline, 202\u00b146 g at 3 years;\n                    <jats:italic>P<\/jats:italic>\n                    for trend &lt;0.001), an improvement in myocardial function (systolic radial strain rate, 2.3\u00b10.4 and 2.9\u00b10.6 seconds\n                    <jats:sup>\u22121<\/jats:sup>\n                    , respectively;\n                    <jats:italic>P<\/jats:italic>\n                    for trend=0.045), and a higher exercise capacity obtained by bicycle stress exercise (106\u00b114 and 122\u00b126 W, respectively;\n                    <jats:italic>P<\/jats:italic>\n                    for trend=0.014). In contrast, patients with mild or severe fibrosis showed a minor reduction in left ventricular hypertrophy and no improvement in myocardial function or exercise capacity.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>\n                      <jats:italic>Conclusions\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    These data suggest that treatment of Fabry cardiomyopathy with recombinant \u03b1-galactosidase A should best be started before myocardial fibrosis has developed to achieve long-term improvement in myocardial morphology and function and exercise capacity.\n                  <\/jats:p>","DOI":"10.1161\/circulationaha.108.794529","type":"journal-article","created":{"date-parts":[[2009,1,19]],"date-time":"2009-01-19T21:24:49Z","timestamp":1232400289000},"page":"524-529","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":431,"title":["Long-Term Effects of Enzyme Replacement Therapy on Fabry Cardiomyopathy"],"prefix":"10.1161","volume":"119","author":[{"given":"Frank","family":"Weidemann","sequence":"first","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Niemann","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Breunig","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Herrmann","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meinrad","family":"Beer","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Sto\u0308rk","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfram","family":"Voelker","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georg","family":"Ertl","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Wanner","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u0308rg","family":"Strotmann","sequence":"additional","affiliation":[{"name":"From the Department of Medicine, Divisions of Cardiology and Nephrology (F.W., M.N., F.B., S.H., S.S., W.V., G.E., C.W., J.S.) and Department of Radiology (M.B.), University Hospital, Wuerzburg, Germany."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-138-4-200302180-00014"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107053450102"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.285.21.2743"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.cir.0000091253.71282.04"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehi143"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2005.11.087"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.105.4.539"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ehj.2003.09.017"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2004.01424.x"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2006.104026"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000237866.70357.c6"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.cir.0000061952.27445.a0"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehm454"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00025.2002"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-146-2-200701160-00148"}],"container-title":["Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCULATIONAHA.108.794529","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T01:51:43Z","timestamp":1715565103000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/CIRCULATIONAHA.108.794529"}},"subtitle":["Evidence for a Better Outcome With Early Treatment"],"short-title":[],"issued":{"date-parts":[[2009,2,3]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2009,2,3]]}},"alternative-id":["10.1161\/CIRCULATIONAHA.108.794529"],"URL":"https:\/\/doi.org\/10.1161\/circulationaha.108.794529","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.718240419.793489689","asserted-by":"object"}]},"ISSN":["0009-7322","1524-4539"],"issn-type":[{"value":"0009-7322","type":"print"},{"value":"1524-4539","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,2,3]]},"assertion":[{"value":"2009-01-19","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}