{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:00:51Z","timestamp":1791295251818,"version":"4.1.0"},"reference-count":42,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"7","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Cardiovascular Imaging"],"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:sec>\n            <jats:title>Background:<\/jats:title>\n            <jats:p>Fabry disease (FD) is an X-linked lysosomal storage disease resulting in tissue accumulation of sphingolipids. Key myocardial processes that lead to adverse outcomes in FD include storage, hypertrophy, inflammation, and fibrosis. These are quantifiable by multiparametric cardiovascular magnetic resonance. Recent developments in cardiovascular magnetic resonance perfusion mapping allow rapid in-line perfusion quantification permitting broader clinical application, including the assessment of microvascular dysfunction. We hypothesized that microvascular dysfunction in FD would be associated with storage, fibrosis, and edema.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods:<\/jats:title>\n            <jats:p>A prospective, observational study of 44 FD patients (49 years, 43% male, 24 [55%] with left ventricular hypertrophy [LVH]) and 27 healthy controls with multiparametric cardiovascular magnetic resonance including vasodilator stress perfusion mapping. Myocardial blood flow (MBF) was measured and its associations with other processes investigated.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results:<\/jats:title>\n            <jats:p>\n              Compared with LVH\u2212 FD, LVH+ FD had higher left ventricular ejection fraction (73% versus 68%), more late gadolinium enhancement (85% versus 15%), and a lower stress MBF (1.76 versus 2.36 mL\/g per minute). The reduction in stress MBF was more pronounced in the subendocardium than subepicardium. LVH\u2212 FD had lower stress MBF than controls (2.36 versus 3.00 mL\/g per minute;\n              <jats:italic>P<\/jats:italic>\n              =0.002). Across all FD, late gadolinium enhancement and low native T1 were independently associated with reduced stress MBF. On a per-segment basis, stress MBF was independently associated with wall thickness, T2, extracellular volume fraction, and late gadolinium enhancement.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions:<\/jats:title>\n            <jats:p>FD patients have reduced perfusion, particularly in the subendocardium with greater reductions with LVH, storage, edema, and scar. Perfusion is reduced even without LVH suggesting it is an early disease marker.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circimaging.119.008872","type":"journal-article","created":{"date-parts":[[2019,7,4]],"date-time":"2019-07-04T13:59:56Z","timestamp":1562248796000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":57,"title":["Quantitative Myocardial Perfusion in Fabry Disease"],"prefix":"10.1161","volume":"12","author":[{"given":"Kristopher D.","family":"Knott","sequence":"first","affiliation":[{"name":"Institute of Cardiovascular Science, University College London, United Kingdom (K.D.K., J.B.A., S.N., R.K.H., C.M., J.C.M.)."},{"name":"Advanced Cardiac Imaging, Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit and The Inherited Cardiovascular Diseases Unit, St Bartholomew\u2019s Hospital, West Smithfield, London, United Kingdom (K.D.K., J.B.A., S.N., C.C., R.K.H., C.M., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joao B.","family":"Augusto","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London, United Kingdom (K.D.K., J.B.A., S.N., R.K.H., C.M., J.C.M.)."},{"name":"Advanced Cardiac Imaging, Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit and The Inherited Cardiovascular Diseases Unit, St Bartholomew\u2019s Hospital, West Smithfield, London, United Kingdom (K.D.K., J.B.A., S.N., C.C., R.K.H., C.M., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Nordin","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London, United Kingdom (K.D.K., J.B.A., S.N., R.K.H., C.M., J.C.M.)."},{"name":"Advanced Cardiac Imaging, Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit and The Inherited Cardiovascular Diseases Unit, St Bartholomew\u2019s Hospital, West Smithfield, London, United Kingdom (K.D.K., J.B.A., S.N., C.C., R.K.H., C.M., 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":"Claudia","family":"Camaioni","sequence":"additional","affiliation":[{"name":"Advanced Cardiac Imaging, Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit and The Inherited Cardiovascular Diseases Unit, St Bartholomew\u2019s Hospital, West Smithfield, London, United Kingdom (K.D.K., J.B.A., S.N., C.C., R.K.H., C.M., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui","family":"Xue","sequence":"additional","affiliation":[{"name":"Medical Signal and Image Processing, National Heart, Lung, and Blood Institute, National Institutes of Health, DHHS, Bethesda, MD (H.X., P.K.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca K.","family":"Hughes","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London, United Kingdom (K.D.K., J.B.A., S.N., R.K.H., C.M., J.C.M.)."},{"name":"Advanced Cardiac Imaging, Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit and The Inherited Cardiovascular Diseases Unit, St Bartholomew\u2019s Hospital, West Smithfield, London, United Kingdom (K.D.K., J.B.A., S.N., C.C., R.K.H., C.M., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"Manisty","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London, United Kingdom (K.D.K., J.B.A., S.N., R.K.H., C.M., J.C.M.)."},{"name":"Advanced Cardiac Imaging, Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit and The Inherited Cardiovascular Diseases Unit, St Bartholomew\u2019s Hospital, West Smithfield, London, United Kingdom (K.D.K., J.B.A., S.N., C.C., R.K.H., C.M., J.C.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise A.E.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Biomedical Imaging Science, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Clarendon Way, United Kingdom (L.A.E.B., S.P.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Kellman","sequence":"additional","affiliation":[{"name":"Medical Signal and Image Processing, National Heart, Lung, and Blood Institute, National Institutes of Health, DHHS, Bethesda, MD (H.X., P.K.)."}],"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., 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