{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T19:50:51Z","timestamp":1769629851397,"version":"3.49.0"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2019,3,17]],"date-time":"2019-03-17T00:00:00Z","timestamp":1552780800000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>Anderson\u2013Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with multi-organ dysfunction. While native myocardial T1 mapping by magnetic resonance (MR) allow non-invasive measurement of myocyte sphingolipid accumulation, 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) and MR are able to identify different pathological patterns of disease progression. We investigated the relationship between T1 mapping and 18F-FDG uptake by hybrid PET-MR cardiac imaging in AFD female patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>Twenty AFD females without cardiac symptoms underwent cardiac PET-MR using 18F-FDG for glucose uptake. In all patients and in seven age- and sex-matched control subjects, T1 mapping was performed using native T1 Modified Look-Locker Inversion-recovery prototype sequences. 18F-FDG myocardial uptake was quantified by measuring the coefficient of variation (COV) of the standardized uptake value using a 17-segment model. T1 values of AFD patients were lower compared with control subjects (1236\u2009\u00b1\u200949\u2009ms vs. 1334\u2009\u00b1\u200927\u2009ms, P\u2009&lt;\u20090.0001). Focal 18F-FDG uptake with COV &gt;0.17 was detected in seven patients. COV was 0.32\u2009\u00b1\u20090.1 in patients with focal 18F-FDG uptake and 0.12\u2009\u00b1\u20090.04 in those without (P\u2009&lt;\u20090.001). Patients with COV &gt;0.17 had higher T1 values of lateral segments of the mid ventricular wall, compared with those with COV \u22640.17 (1216\u2009\u00b1\u200922\u2009ms vs. 1160\u2009\u00b1\u200959\u2009ms, P\u2009&lt;\u20090.05).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In females with AFD, focal 18F-FDG uptake with a trend towards a pseudo-normalization of abnormal T1 mapping values, may represent an intermediate stage before the development of myocardial fibrosis. These findings suggest a potential relationship between progressive myocyte sphingolipid accumulation and inflammation.<\/jats:p><\/jats:sec>","DOI":"10.1093\/ehjci\/jez039","type":"journal-article","created":{"date-parts":[[2019,2,25]],"date-time":"2019-02-25T20:13:27Z","timestamp":1551125607000},"page":"1004-1011","source":"Crossref","is-referenced-by-count":29,"title":["Hybrid positron emission tomography-magnetic resonance imaging for assessing different stages of cardiac impairment in patients with Anderson\u2013Fabry disease: AFFINITY study group"],"prefix":"10.1093","volume":"20","author":[{"given":"Massimo","family":"Imbriaco","sequence":"first","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmela","family":"Nappi","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Ponsiglione","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Pisani","sequence":"additional","affiliation":[{"name":"Department of Public Health, University of Naples Federico II, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Serena","family":"Dell\u2019Aversana","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuele","family":"Nicolai","sequence":"additional","affiliation":[{"name":"SDN IRCCS, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Letizia","family":"Spinelli","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Aiello","sequence":"additional","affiliation":[{"name":"SDN IRCCS, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudio Tommaso","family":"Diomiaiuti","sequence":"additional","affiliation":[{"name":"SDN IRCCS, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eleonora","family":"Riccio","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberta","family":"Esposito","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maurizio","family":"Galderisi","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariangela","family":"Losi","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Greiser","sequence":"additional","affiliation":[{"name":"Siemens Healthcare, Erlangen, 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