{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T08:00:35Z","timestamp":1792569635191,"version":"4.5.0"},"reference-count":28,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"11","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2025,6,3]]},"abstract":"<jats:sec id=\"jah310874-sec-0001\" xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Fabry disease can be classified in 4 stages based on the extent of cardiac damage assessed with echocardiography. This staging is strongly associated with prognosis, with a doubled risk increase of cardiovascular events for each stage progression. The aims of the present study were to investigate the evolution of cardiac damage using this staging system during midterm follow\u2010up and to identify predictors of stage worsening.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"jah310874-sec-0002\" xml:lang=\"en\">\n                    <jats:title>Methods AND RESULTS<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      This is a retrospective, multicenter study, which included 168 patients with Fabry disease (38% men) categorized at baseline and follow\u2010up in 4 stages, defined as follows: stage 0, no cardiac involvement; stage 1, left ventricular hypertrophy; stage 2, left atrial enlargement; and stage 3, ventricular impairment. After a median of 5\u2009years, cardiac stage worsened of at least 1 class in 34 patients (24% of 143 patients in stage 0\u20132 at baseline), improved in 9 (12% of 73 patients in stages 1\u20133 at baseline), and remained stable in 125 (74% of the 168 patients enrolled). At multivariable logistic regression analysis, age (odds ratio, 1.05 [95% CI, 1.01\u20131.09];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.006), male sex (odds ratio, 4.26 [95% CI, 1.54\u201311.79];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.005), classic phenotype (odds ratio, 4.95 [95% CI, 1.30\u201318.72];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.018), and renal failure (odds ratio, 10.40 [95% CI, 1.57\u201368.77];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.015) emerged as independent predictors of cardiac damage stage deterioration.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"jah310874-sec-0004\" xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Fabry disease is associated with a high rate of progression of cardiac damage during midterm follow\u2010up, with \u224824% of patients experiencing cardiac stage worsening. Age, male sex, classic phenotype, and renal failure are independent predictors of stage progression.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.124.041627","type":"journal-article","created":{"date-parts":[[2025,5,23]],"date-time":"2025-05-23T06:49:13Z","timestamp":1747982953000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":12,"title":["Evolution of Cardiac Damage Staged With Echocardiography in Fabry Disease"],"prefix":"10.1161","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0375-5949","authenticated-orcid":false,"given":"Rosa","family":"Lillo","sequence":"first","affiliation":[{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS","place":["Rome, Italy"]},{"name":"Universit\u00e0 Cattolica del Sacro Cuore","place":["Rome, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5474-0555","authenticated-orcid":false,"given":"Maria Chiara","family":"Meucci","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS","place":["Rome, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1405-9436","authenticated-orcid":false,"given":"Annamaria","family":"Del Franco","sequence":"additional","affiliation":[{"name":"University of Florence","place":["Florence, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9304-988X","authenticated-orcid":false,"given":"Emanuele","family":"Monda","sequence":"additional","affiliation":[{"name":"University of Campania \u201cLuigi Vanvitelli\u201d, Monaldi Hospital","place":["Naples, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Iannaccone","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS","place":["Rome, Italy"]},{"name":"Universit\u00e0 Cattolica del Sacro Cuore","place":["Rome, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7025-0679","authenticated-orcid":false,"given":"Raffaello","family":"Ditaranto","sequence":"additional","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna","place":["Bologna, Italy"]},{"name":"European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart\u2010ERN GUARD\u2010Heart"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-4491-3669","authenticated-orcid":false,"given":"Federico","family":"Di Nicola","sequence":"additional","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna","place":["Bologna, Italy"]},{"name":"European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart\u2010ERN GUARD\u2010Heart"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serena","family":"Serratore","sequence":"additional","affiliation":[{"name":"Magna Graecia University","place":["Catanzaro, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3162-1830","authenticated-orcid":false,"given":"Antonella","family":"Lombardo","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario A. 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