{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T04:47:18Z","timestamp":1768538838224,"version":"3.49.0"},"reference-count":22,"publisher":"Cambridge University Press (CUP)","issue":"3","license":[{"start":{"date-parts":[[2023,8,31]],"date-time":"2023-08-31T00:00:00Z","timestamp":1693440000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Noonan syndrome is a multi-system genetic disorder and patients may suffer from hypertrophic cardiomyopathy. Previous studies have identified electrocardiographic features that may support a diagnosis of Noonan syndrome. In this two-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 30 patients with Noonan syndrome with hypertrophic cardiomyopathy and compared these with the electrocardiographic features in 15 children with sarcomeric hypertrophic cardiomyopathy. Typical Noonan syndrome-related electrocardiographic features are a negative aVF, small left precordial R-waves, large right precordial S-waves, and abnormal Q-wave. We also analysed electrocardiographic features of hypertrophic cardiomyopathy: ST-segment abnormalities and T-wave abnormalities. A negative aVF was seen in 83% of patients with Noonan syndrome-related hypertrophic cardiomyopathy in contrast to 27% of patients with primary sarcomeric hypertrophic cardiomyopathy (p &lt; 0.001). An extreme QRS axis in the north-west was seen only in patients with Noonan syndrome-related hypertrophic cardiomyopathy. This QRS axis deviation is likely to be determined by the Noonan syndrome-related hypertrophic cardiomyopathy and not by the type of hypertrophic cardiomyopathy. There were no differences between the two groups in the frequency of large right precordial S-waves and small R-waves in the left precordial leads V5 and V6. However, an abnormal R\/S ratio was more often seen in patients with Noonan syndrome-related hypertrophic cardiomyopathy (p &lt; 0.001). Pathologic Q-waves were seen statistically more frequently in patients with sarcomeric hypertrophic cardiomyopathy (p = 0.009). The occurrence of ST-segment and T-wave pathology did not statistically differ between the two groups. Electrography can be of use in differentiating sarcomeric hypertrophic cardiomyopathy from Noonan syndrome-related hypertrophic cardiomyopathy.<\/jats:p>","DOI":"10.1017\/s1047951123003177","type":"journal-article","created":{"date-parts":[[2023,8,31]],"date-time":"2023-08-31T07:15:11Z","timestamp":1693466111000},"page":"597-603","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":2,"title":["Differentiating primary sarcomeric hypertrophic cardiomyopathy from Noonan syndrome: can the electrocardiogram be of use?"],"prefix":"10.1017","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8544-779X","authenticated-orcid":false,"given":"Robert W. L.","family":"Hauptmeijer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lea","family":"Lippert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Floris E. A. Udink","family":"ten Cate","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zina","family":"Fejzic","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika","family":"Leenders","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4701-5843","authenticated-orcid":false,"given":"Cordula M.","family":"Wolf","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4829-6173","authenticated-orcid":false,"given":"Jos M. T.","family":"Draaisma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2023,8,31]]},"reference":[{"key":"S1047951123003177_ref8","doi-asserted-by":"publisher","DOI":"10.21037\/cdt.2019.05.01"},{"key":"S1047951123003177_ref22","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-0736(99)90109-7"},{"key":"S1047951123003177_ref4","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951121003310"},{"key":"S1047951123003177_ref13","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32140"},{"key":"S1047951123003177_ref9","doi-asserted-by":"crossref","first-page":"2733","DOI":"10.1093\/eurheartj\/ehu284","article-title":"ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)","volume":"35","author":"Elliott","year":"2014","journal-title":"Eur Heart J"},{"key":"S1047951123003177_ref18","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-323-16951-6.00003-4"},{"key":"S1047951123003177_ref21","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmg.2021.104372"},{"key":"S1047951123003177_ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.ppedcard.2015.01.002"},{"key":"S1047951123003177_ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.hfc.2021.07.004"},{"key":"S1047951123003177_ref6","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehy798"},{"key":"S1047951123003177_ref3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.05.024"},{"key":"S1047951123003177_ref5","doi-asserted-by":"publisher","DOI":"10.1111\/chd.12102"},{"key":"S1047951123003177_ref17","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2015.01.025"},{"key":"S1047951123003177_ref2","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2021-322858"},{"key":"S1047951123003177_ref14","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-008-0670-9"},{"key":"S1047951123003177_ref19","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2006.09.001"},{"key":"S1047951123003177_ref15","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-2-4"},{"key":"S1047951123003177_ref1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.23624"},{"key":"S1047951123003177_ref16","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezx369"},{"key":"S1047951123003177_ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2012.04.018"},{"key":"S1047951123003177_ref10","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/cvv024"},{"key":"S1047951123003177_ref12","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehs397"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951123003177","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T10:58:31Z","timestamp":1709895511000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951123003177\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8,31]]},"references-count":22,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["S1047951123003177"],"URL":"https:\/\/doi.org\/10.1017\/s1047951123003177","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"value":"1047-9511","type":"print"},{"value":"1467-1107","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,8,31]]},"assertion":[{"value":"\u00a9 The Author(s), 2023. 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