{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T17:32:58Z","timestamp":1790962378881,"version":"4.1.0"},"reference-count":32,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"12","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Cardiovascular Imaging"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Cardiac response to enzyme replacement therapy (ERT) in Fabry disease is typically assessed by measuring left ventricular mass index using echocardiography or cardiovascular magnetic resonance, but neither quantifies myocardial biology. Low native T1 in Fabry disease represents sphingolipid accumulation; late gadolinium enhancement with high T2 and troponin elevation reflects inflammation. We evaluated the effect of ERT on myocardial storage, inflammation, and hypertrophy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Twenty patients starting ERT (60% left ventricular hypertrophy\u2013positive) were compared with 18 patients with early disease and 18 with advanced disease over 1 year at 3 centers. Cardiovascular magnetic resonance (left ventricular mass index, T1, T2, global longitudinal strain, and late gadolinium enhancement) and biomarkers (high-sensitive troponin-T and NT-proBNP [N-terminal Pro-B-type natriuretic peptide]) at baseline (pre-ERT) and 12 months were performed. Early disease controls were stable, treatment-na\u00efve patients (mainly left ventricular hypertrophy\u2013negative); advanced disease controls were stable, established ERT patients (mainly left ventricular hypertrophy\u2013positive).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Over 1 year, early disease controls increased maximum wall thickness and left ventricular mass index (9.8\u00b12.7 versus 10.2\u00b12.6 mm;\n                      <jats:italic>P<\/jats:italic>\n                      =0.010; 65\u00b115 versus 67\u00b116 g\/m\n                      <jats:sup>2<\/jats:sup>\n                      ;\n                      <jats:italic>P<\/jats:italic>\n                      =0.005) and native T1 fell (981\u00b158 versus 959\u00b161 ms;\n                      <jats:italic>P<\/jats:italic>\n                      =0.002). Advanced disease controls increased T2 in the late gadolinium enhancement area (57\u00b16 versus 60\u00b17 ms;\n                      <jats:italic>P<\/jats:italic>\n                      =0.023) with worsening global longitudinal strain (\u221213.2\u00b13.4 versus \u221212.1\u00b14.8;\n                      <jats:italic>P<\/jats:italic>\n                      =0.039). Newly treated patients had a small reduction in maximum wall thickness (14.8\u00b15.9 versus 14.4\u00b15.7 mm;\n                      <jats:italic>P<\/jats:italic>\n                      =0.028), stable left ventricular mass index (93\u00b142 versus 92\u00b140 g\/m\n                      <jats:sup>2<\/jats:sup>\n                      ;\n                      <jats:italic>P<\/jats:italic>\n                      =0.186) and a reduction in T1 lowering (917\u00b149 versus 931\u00b154 ms;\n                      <jats:italic>P<\/jats:italic>\n                      =0.017).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Fabry myocardial phenotype development is different at different disease stages. After 1 year of ERT initiation, left ventricular hypertrophy\u2013positive patients have a detectable, small reduction in left ventricular mass and storage.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circimaging.119.009430","type":"journal-article","created":{"date-parts":[[2019,12,12]],"date-time":"2019-12-12T05:00:27Z","timestamp":1576126827000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":71,"title":["Myocardial Storage, Inflammation, and Cardiac Phenotype in Fabry Disease After One Year of Enzyme Replacement Therapy"],"prefix":"10.1161","volume":"12","author":[{"given":"Sabrina","family":"Nordin","sequence":"first","affiliation":[{"name":"From the Institute of Cardiovascular Science, University College London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."},{"name":"Cardiology Department, Barts Heart Centre, London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Kozor","sequence":"additional","affiliation":[{"name":"Sydney Medical School, University of Sydney, Australia (R.K.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ravi","family":"Vijapurapu","sequence":"additional","affiliation":[{"name":"Cardiology Department (R.V., R.P.S.), University Hospitals Birmingham, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o B.","family":"Augusto","sequence":"additional","affiliation":[{"name":"From the Institute of Cardiovascular Science, University College London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."},{"name":"Cardiology Department, Barts Heart Centre, London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristopher D.","family":"Knott","sequence":"additional","affiliation":[{"name":"From the Institute of Cardiovascular Science, University College London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."},{"name":"Cardiology Department, Barts Heart Centre, London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriella","family":"Captur","sequence":"additional","affiliation":[{"name":"From the Institute of Cardiovascular Science, University College London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."},{"name":"Cardiology Department, Barts Heart Centre, London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas A.","family":"Treibel","sequence":"additional","affiliation":[{"name":"From the Institute of Cardiovascular Science, University College London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."},{"name":"Cardiology Department, Barts Heart Centre, London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uma","family":"Ramaswami","sequence":"additional","affiliation":[{"name":"Lysosomal Storage Disorder Unit, Royal Free Hospital, London, United Kingdom (U.R., D.A.H.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Tchan","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine, Westmead Hospital, Sydney, Australia (M.T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tarekegn","family":"Geberhiwot","sequence":"additional","affiliation":[{"name":"Inherited Metabolic Disorders Unit (T.G.), University Hospitals Birmingham, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard P.","family":"Steeds","sequence":"additional","affiliation":[{"name":"Cardiology Department (R.V., R.P.S.), University Hospitals Birmingham, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derralynn A.","family":"Hughes","sequence":"additional","affiliation":[{"name":"Lysosomal Storage Disorder Unit, Royal Free Hospital, London, United Kingdom (U.R., D.A.H.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James C.","family":"Moon","sequence":"additional","affiliation":[{"name":"From the Institute of Cardiovascular Science, University College London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., J.C.M.)."},{"name":"Cardiology Department, Barts Heart Centre, London, United Kingdom (S.N., J.B.A., K.D.K., G.C., T.A.T., 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