{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T02:54:22Z","timestamp":1768791262099,"version":"3.49.0"},"reference-count":40,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2018,9,24]],"date-time":"2018-09-24T00:00:00Z","timestamp":1537747200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007397","name":"Univerzita Karlova v Praze","doi-asserted-by":"publisher","award":["PROGRES Q38\/LF1"],"award-info":[{"award-number":["PROGRES Q38\/LF1"]}],"id":[{"id":"10.13039\/100007397","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Echocardiography"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Fabry cardiomyopathy (FC) and light\u2010chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy\/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A two\u2010center echocardiographic analysis was performed, comprising 118 patients with IVS \u226512\u00a0mm (FC and AL 59 patients each) matched by IVS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fabry cardiomyopathy patients had larger LV end\u2010diastolic diameter (47.7 [44.0\u201350.9] vs 45.0 [41.5\u201349.0]\u00a0mm, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.002), better LV ejection fraction (EF 68.7 [63.4\u201374.0] vs 63.0 [54.0\u201370.0]%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.001) and midwall fractional shortening (midFS 14.8 [13.0\u201316.1] vs 12.1 [8.9\u201315.0]%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.006). LV EF &lt;40% was rare in both (2% vs 7%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.004), with higher E\/e\u2019 ratio (13.6 [10.2\u201318.8] vs 9.8 [7.5\u201312.3], <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.0001). Average E\/e\u2019 ratio and midFS were significantly associated with NYHA severity in both groups (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.05 for all).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Matched AL patients had worse LV diastolic function than FC, driven by E\/e\u2019. Significant LV systolic dysfunction was rare overall. MidFS and E\/e\u2019 were associated with heart failure severity in both groups.<\/jats:p><\/jats:sec>","DOI":"10.1111\/echo.14144","type":"journal-article","created":{"date-parts":[[2018,9,24]],"date-time":"2018-09-24T12:37:38Z","timestamp":1537792658000},"page":"1755-1763","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Comparison of echocardiographic parameters in Fabry cardiomyopathy and light\u2010chain cardiac amyloidosis"],"prefix":"10.1111","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1547-5696","authenticated-orcid":false,"given":"Josef","family":"Marek","sequence":"first","affiliation":[{"name":"2nd Department of Medicine\u2014Department of Cardiovascular Medicine First Faculty of Medicine Charles University and General University Hospital in Prague  Prague Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomas","family":"Palecek","sequence":"additional","affiliation":[{"name":"2nd Department of Medicine\u2014Department of Cardiovascular Medicine First Faculty of Medicine Charles University and General University Hospital in Prague  Prague Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julien","family":"Magne","sequence":"additional","affiliation":[{"name":"Department of Cardiology Dupuytren University Hospital  Limoges France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Lavergne","sequence":"additional","affiliation":[{"name":"Department of Hematology National Reference Center for Light\u2010chain Systemic Amyloidosis Dupuytren University Hospital  Limoges France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cyrille","family":"Boulogne","sequence":"additional","affiliation":[{"name":"Department of Cardiology Dupuytren University Hospital  Limoges France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bahaa M.","family":"Fadel","sequence":"additional","affiliation":[{"name":"Section of Adult Cardiology Heart Center King Faisal Specialist Hospital &amp; Research Center  Riyadh Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arnaud","family":"Jaccard","sequence":"additional","affiliation":[{"name":"Department of Hematology National Reference Center for Light\u2010chain Systemic Amyloidosis Dupuytren University Hospital  Limoges France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ales","family":"Linhart","sequence":"additional","affiliation":[{"name":"2nd Department of Medicine\u2014Department of Cardiovascular Medicine First Faculty of Medicine Charles University and General University Hospital in Prague  Prague Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dania","family":"Mohty","sequence":"additional","affiliation":[{"name":"Department of Cardiology Dupuytren University Hospital  Limoges France"},{"name":"Section of Adult Cardiology Heart Center King Faisal Specialist Hospital &amp; Research Center  Riyadh Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,9,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.06.053"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20381"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.acvd.2013.06.051"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2014.104109"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-5-30"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2005.063818"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3181bb05bb"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107053450102"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.285.21.2743"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0338-2"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(89)90254-4"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00380-005-0852-6"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/ehjci\/jev014"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/4.suppl_G.1"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.62.3.548"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.843334"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.44.4.548"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(94)90390-5"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/HJH.0000000000000120"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2010.05.010"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1093\/ehjci\/jew082"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.70.6.726"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(77)80189-6"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1067\/mhj.2000.105105"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehx043"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1012428009627"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jjcc.2007.12.001"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(85)80475-7"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.93.2.259"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(09)61493-8"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.66.4.790"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.83.3.808"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01606034"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2012.684810"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1161\/circ.104.14.1594"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00503.2011"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000068313.67758.4F"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.117.006588"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2014.01.013"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.117.007168"}],"container-title":["Echocardiography"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fecho.14144","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/echo.14144","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T10:30:16Z","timestamp":1694860216000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/echo.14144"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,24]]},"references-count":40,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2018,11]]}},"alternative-id":["10.1111\/echo.14144"],"URL":"https:\/\/doi.org\/10.1111\/echo.14144","archive":["Portico"],"relation":{},"ISSN":["0742-2822","1540-8175"],"issn-type":[{"value":"0742-2822","type":"print"},{"value":"1540-8175","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,24]]},"assertion":[{"value":"2018-06-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-08-25","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-09-24","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}