{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T15:43:00Z","timestamp":1777390980517,"version":"3.51.4"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2017,3,7]],"date-time":"2017-03-07T00:00:00Z","timestamp":1488844800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Echocardiography"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:p>Classic infantile\u2010onset Pompe disease (<jats:styled-content style=\"fixed-case\">IOPD<\/jats:styled-content>), characterized by predominantly cardiac involvement, used to be considered uniformly lethal within months. The availability of enzyme replacement therapy (<jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>) has transformed the course of the disease. Decrease in ventricular hypertrophy and improvement in ventricular function have been suggested as proof for efficacy. We report the cardiac response to <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content> of a child with <jats:styled-content style=\"fixed-case\">IOPD<\/jats:styled-content> and severe hypertrophic cardiomyopathy. The myocardial hypertrophy resolved. Change in ejection fraction, however, was slow. We discuss the potential benefit of other parameters beyond ejection to assess cardiac function in <jats:styled-content style=\"fixed-case\">IOPD<\/jats:styled-content>, including speckle tracking\u2010based strain.<\/jats:p>","DOI":"10.1111\/echo.13490","type":"journal-article","created":{"date-parts":[[2017,3,7]],"date-time":"2017-03-07T12:06:43Z","timestamp":1488888403000},"page":"621-624","source":"Crossref","is-referenced-by-count":8,"title":["Cardiac response to enzyme replacement therapy in infantile Pompe disease with severe hypertrophic cardiomyopathy"],"prefix":"10.1111","volume":"34","author":[{"given":"Sravani","family":"Avula","sequence":"first","affiliation":[{"name":"Department of Pediatrics (Cardiology) Louisiana State University Health Sciences Center  New Orleans LA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thuylinh M.","family":"Nguyen","sequence":"additional","affiliation":[{"name":"School of Medicine Louisiana State University Health Sciences Center  New Orleans LA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Marble","sequence":"additional","affiliation":[{"name":"Department of Pediatrics (Genetics) Louisiana State University Health Sciences Center  New Orleans LA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3799-4689","authenticated-orcid":false,"given":"Christian","family":"Lilje","sequence":"additional","affiliation":[{"name":"Department of Pediatrics (Cardiology) Louisiana State University Health Sciences Center  New Orleans LA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,3,7]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2014.12.004"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pedneo.2013.03.009"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-9-75"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/cvv025"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.112.2.332"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cardfail.2011.07.011"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.741157"},{"key":"e_1_2_5_9_1","volume-title":"Measurements in cardiology","author":"Sutton P","year":"1999"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2003.12.005"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951104002070"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.112.000175"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12947-015-0029-0"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2008.08.011"},{"key":"e_1_2_5_15_1","first-page":"304","article-title":"Over idiopatische hypertrophie van het hart","volume":"67","author":"Pompe JC","year":"1932","journal-title":"Ned Tijdshr Geneeskd"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.11.033"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e318183722f"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9179-2"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3182142966"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-008-9267-3"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00577.2005"},{"key":"e_1_2_5_22_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0067052"},{"key":"e_1_2_5_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-014-9707-6"}],"container-title":["Echocardiography"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fecho.13490","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/echo.13490","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T21:42:53Z","timestamp":1695505373000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/echo.13490"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,3,7]]},"references-count":22,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2017,4]]}},"alternative-id":["10.1111\/echo.13490"],"URL":"https:\/\/doi.org\/10.1111\/echo.13490","archive":["Portico"],"relation":{},"ISSN":["0742-2822","1540-8175"],"issn-type":[{"value":"0742-2822","type":"print"},{"value":"1540-8175","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,3,7]]}}}