{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T14:07:58Z","timestamp":1793196478339,"version":"4.5.0"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2023,6,13]],"date-time":"2023-06-13T00:00:00Z","timestamp":1686614400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"University Hospital in Prague"},{"DOI":"10.13039\/100007397","name":"Charles University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007397","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,23]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Fabry disease (FD) is a multisystemic lysosomal storage disorder caused by a defect in the alpha-galactosidase A gene that manifests as a phenocopy of hypertrophic cardiomyopathy. We assessed the echocardiographic 3D left ventricular (LV) strain of patients with FD in relation to heart failure severity using natriuretic peptides, the presence of a cardiovascular magnetic resonance (CMR) late gadolinium enhancement scar, and long-term prognosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>3D echocardiography was feasible in 75\/99 patients with FD [aged 47 \u00b1 14 years, 44% males, LV ejection fraction (EF) 65 \u00b1 6% and 51% with hypertrophy or concentric remodelling of the LV]. Long-term prognosis (death, heart failure decompensation, or cardiovascular hospitalization) was assessed over a median follow-up of 3.1 years. A stronger correlation was observed for N-terminal pro-brain natriuretic peptide levels with 3D LV global longitudinal strain (GLS, r = \u22120.49, P &amp;lt; 0.0001) than with 3D LV global circumferential strain (GCS, r = \u22120.38, P &amp;lt; 0.001) or 3D LVEF (r = \u22120.25, P = 0.036). Individuals with posterolateral scar on CMR had lower posterolateral 3D circumferential strain (CS; P = 0.009). 3D LV-GLS was associated with long-term prognosis [adjusted hazard ratio 0.85 (confidence interval 0.75\u20130.95), P = 0.004], while 3D LV-GCS and 3D LVEF were not (P = 0.284 and P = 0.324).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>3D LV-GLS is associated with both heart failure severity measured by natriuretic peptide levels and long-term prognosis. Decreased posterolateral 3D CS reflects typical posterolateral scarring in FD. Where feasible, 3D-strain echocardiography can be used for a comprehensive mechanical assessment of the LV in patients with FD.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjci\/jead121","type":"journal-article","created":{"date-parts":[[2023,6,13]],"date-time":"2023-06-13T10:19:25Z","timestamp":1686651565000},"page":"1629-1637","source":"Crossref","is-referenced-by-count":11,"title":["Three-dimensional echocardiographic left ventricular strain analysis in Fabry disease: correlation with heart failure severity, myocardial scar, and impact on long-term prognosis"],"prefix":"10.1093","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1547-5696","authenticated-orcid":false,"given":"Josef","family":"Marek","sequence":"first","affiliation":[{"name":"2nd Department of Medicine \u2013 Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital , U nemocnice 2, Prague 2, 128 02 , Czech 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Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital , U nemocnice 2, Prague 2, 128 02 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3372-7850","authenticated-orcid":false,"given":"Ale\u0161","family":"Linhart","sequence":"additional","affiliation":[{"name":"2nd Department of Medicine \u2013 Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital , U nemocnice 2, Prague 2, 128 02 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,6,13]]},"reference":[{"key":"2023112322342813800_jead121-B1","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.ymgme.2018.02.014","article-title":"Fabry disease revisited: management and treatment recommendations for adult 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