{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T14:12:48Z","timestamp":1768486368001,"version":"3.49.0"},"reference-count":42,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,1,19]],"date-time":"2020-01-19T00:00:00Z","timestamp":1579392000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Echocardiography"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Fabry disease (FD) is a rare X\u2010linked storage disorder caused by deficiency of the lysosomal enzyme \u03b1\u2010galactosidase A, and it typically causes multiorgan dysfunction. The main cause of death is heart disease resulting from left ventricular (LV) diastolic dysfunction, LV systolic dysfunction, severe LV hypertrophy (LVH), and sudden death. In several cardiac disorders, LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD). MD has until now not been studied in patients with FD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To investigate the prevalence of MD in patients with FD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Complete echocardiographic data and speckle tracking echocardiographic data were collected. MD is an index of inter\u2010segmental discoordination of contraction and is defined as the standard deviation (SD) of the time\u2010to\u2010peak longitudinal negative strain in 17 LV segments with a value &gt;49 milliseconds. Patients with FD were divided into the following 2 groups: group I (patients with FD but no LVH, n\u00a0=\u00a064) and group II (patients with FD and LVH, n\u00a0=\u00a025). These groups were compared with a group of healthy subjects (group III, n\u00a0=\u00a050). Parametric variables were expressed as mean\u00a0\u00b1\u00a0SD, and nonparametric variables were expressed as median and inter\u2010quartile range. A <jats:italic>P<\/jats:italic> value &lt;.05 was considered significant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 113 patients with FD were included in this study. Of these, 24 (21%) were excluded because of poor imaging quality or presence of comorbidities, and the final study population consisted of 89 patients (mean age of 33.5\u00a0\u00b1\u00a014.5\u00a0years, 64% female). Group II patients were older than group I patients (46\u00a0\u00b1\u00a013\u00a0years vs 27\u00a0\u00b1\u00a011\u00a0years, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.0001). There was no difference in LV ejection fraction between the 3 groups. There was also no difference in MD between groups I and III (32.4\u00a0ms [26\u201039] vs 32\u00a0ms [26\u201039]). In group II, the MD in 19 patients (76%) was 56\u00a0ms (39\u201080).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>To the best of our knowledge, this is the first study to assess the prevalence of MD in patients with FD. MD was observed in 76% of patients with FD and LVH. The use of MD in strain echocardiography may be beneficial in the assessment of patients with FD who develop heart failure.<\/jats:p><\/jats:sec>","DOI":"10.1111\/echo.14592","type":"journal-article","created":{"date-parts":[[2020,1,19]],"date-time":"2020-01-19T17:26:25Z","timestamp":1579454785000},"page":"293-301","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Mechanical dispersion in Fabry disease assessed with speckle tracking echocardiography"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5815-1173","authenticated-orcid":false,"given":"Tomas F.","family":"Cianciulli","sequence":"first","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"},{"name":"Researcher of the Ministry of Health of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda C.","family":"Saccheri","sequence":"additional","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario A.","family":"R\u00edsolo","sequence":"additional","affiliation":[{"name":"Division of Cardiology Medical Center of San Luis San Luis Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jorge A.","family":"Lax","sequence":"additional","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo J.","family":"M\u00e9ndez","sequence":"additional","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luis A.","family":"Morita","sequence":"additional","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin A.","family":"Beck","sequence":"additional","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucia R.","family":"Kazeli\u00e1n","sequence":"additional","affiliation":[{"name":"Echocardiography Laboratory Division of Cardiology \"Dr. Cosme Argerich\" Hospital of the Government of the City of Buenos Aires Buenos Aires Argentina"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,1,19]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.281.3.249"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2013.48"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.2119\/molmed.2012.00002"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1086\/504601"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.38.11.750"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2004.01309.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.38.11.769"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.285.21.2743"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfi152"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2014-102797"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1510198"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2014.01.014"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2016-104178"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2018.02.014"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199102073240607"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehm153"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2005.05.033"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2010.11.018"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2004.035584"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2016-310138"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jjcc.2007.12.001"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/ehjci\/jev173"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13063-019-3425-1"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.acvd.2019.01.002"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2014.10.003"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(86)90771-X"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2004.06.019"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.960377"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.2307\/2533395"},{"issue":"4","key":"e_1_2_9_31_1","first-page":"650","article-title":"Physiological determinants of left ventricular mechanical dispersion: a 2\u2010dimensional speckle tracking echocardiographic study in healthy volunteers","volume":"11","author":"Rodr\u00edguez\u2010Zanella H","year":"2018","journal-title":"JACC Cardiovasc Imaging"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2013.03.005"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2012.02.004"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2018.05.017"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2014.04.029"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2009.11.012"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp588"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)02380-X"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ehj.2003.09.017"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jjcc.2008.10.004"},{"issue":"4","key":"e_1_2_9_41_1","first-page":"134","article-title":"Follow\u2010up of cardiac fabry disease treated by cardiac resynchronization therapy","volume":"1","author":"Fukuda Y","year":"2017","journal-title":"CASE (Phila)"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12551-018-0432-5"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.1002\/ejhf.789"}],"container-title":["Echocardiography"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/echo.14592","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/echo.14592","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/echo.14592","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,24]],"date-time":"2023-08-24T03:26:58Z","timestamp":1692847618000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/echo.14592"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,1,19]]},"references-count":42,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2020,2]]}},"alternative-id":["10.1111\/echo.14592"],"URL":"https:\/\/doi.org\/10.1111\/echo.14592","archive":["Portico"],"relation":{},"ISSN":["0742-2822","1540-8175"],"issn-type":[{"value":"0742-2822","type":"print"},{"value":"1540-8175","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,1,19]]},"assertion":[{"value":"2019-11-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-12-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-01-19","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}