{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T22:56:32Z","timestamp":1769122592887,"version":"3.49.0"},"reference-count":39,"publisher":"Cambridge University Press (CUP)","issue":"6","license":[{"start":{"date-parts":[[2021,8,12]],"date-time":"2021-08-12T00:00:00Z","timestamp":1628726400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Noonan syndrome is a genetic disorder characteried by short stature, typical facial features, developmental delay, and CHD. In this single-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 95 patients with clinically and molecularly confirmed Noonan syndrome. Typical Noonan syndrome-related electrocardiographic features are left axis deviation, small left precordial R-waves, large right precordial S-waves, abnormal Q-wave, and abnormal wide QRS complex. In this representative cohort, CHD was found in 59 patients (62.1%) and typical Noonan syndrome-related electrographic features in 60 patients (63.2%). The typical Noonan syndrome-related electrographic features were also increased over baseline in patients without CHD (41.7%). Of all 95 patients, left axis deviation was seen in 46.3%, small left precordial R-waves in 30.5%, large right precordial S-waves in 5.3%, and abnormal Q-wave and wide QRS complex in 2.1%. There was no significant difference in the frequency of the individual-specific electrographic features between the group with CHD and the group without CHD. However, there were significantly more patients with a small left precordial R-wave in the subgroup with pulmonary stenosis compared to patients without pulmonary stenosis. <jats:italic>Conclusion<\/jats:italic>: Specific Noonan syndrome-related electrographic features are frequently present in patients with Noonan syndrome, also in the absence of CHD. These results suggest that there may be a continuum of cardiac anomalies from overt CHD to milder abnormalities that are only seen on electrocardiogram.<\/jats:p>","DOI":"10.1017\/s1047951121003310","type":"journal-article","created":{"date-parts":[[2021,8,12]],"date-time":"2021-08-12T09:47:57Z","timestamp":1628761677000},"page":"936-943","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":9,"title":["The added value of the electrocardiogram in Noonan syndrome"],"prefix":"10.1017","volume":"32","author":[{"given":"Eefke","family":"Vos","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika","family":"Leenders","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sterre R.","family":"Werkman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Floris E. A.","family":"Udink ten Cate","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":[[2021,8,12]]},"reference":[{"key":"S1047951121003310_ref38","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2012.04.018"},{"key":"S1047951121003310_ref31","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.eurheartj.a061452"},{"key":"S1047951121003310_ref28","doi-asserted-by":"publisher","DOI":"10.1038\/ng1926"},{"key":"S1047951121003310_ref19","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-323-16951-6.00003-4"},{"key":"S1047951121003310_ref36","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2007.03.093"},{"key":"S1047951121003310_ref39","doi-asserted-by":"publisher","DOI":"10.1093\/ehjci\/jet105"},{"key":"S1047951121003310_ref32","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-016-1468-6"},{"key":"S1047951121003310_ref27","doi-asserted-by":"publisher","DOI":"10.1086\/340847"},{"key":"S1047951121003310_ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.05.024"},{"key":"S1047951121003310_ref21","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2006.09.001"},{"key":"S1047951121003310_ref1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2009-3207"},{"key":"S1047951121003310_ref34","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.58.3.402"},{"key":"S1047951121003310_ref22","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2007.051276"},{"key":"S1047951121003310_ref25","doi-asserted-by":"publisher","DOI":"10.1038\/ng1748"},{"key":"S1047951121003310_ref9","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-015-1627-5"},{"key":"S1047951121003310_ref35","doi-asserted-by":"publisher","DOI":"10.4103\/1995-705X.90900"},{"key":"S1047951121003310_ref29","doi-asserted-by":"publisher","DOI":"10.1007\/s40279-016-0609-7"},{"key":"S1047951121003310_ref5","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2016.32"},{"key":"S1047951121003310_ref37","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.38854"},{"key":"S1047951121003310_ref24","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.046300"},{"key":"S1047951121003310_ref30","doi-asserted-by":"publisher","DOI":"10.1016\/0167-5273(89)90312-4"},{"key":"S1047951121003310_ref6","doi-asserted-by":"publisher","DOI":"10.1038\/ng772"},{"key":"S1047951121003310_ref18","doi-asserted-by":"publisher","DOI":"10.1007\/BF02083144"},{"key":"S1047951121003310_ref16","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30598"},{"key":"S1047951121003310_ref7","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32140"},{"key":"S1047951121003310_ref12","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320530213"},{"key":"S1047951121003310_ref33","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000669"},{"key":"S1047951121003310_ref17","doi-asserted-by":"publisher","DOI":"10.1007\/s10038-008-0343-6"},{"key":"S1047951121003310_ref15","doi-asserted-by":"publisher","DOI":"10.1590\/S0066-782X2000001100005"},{"key":"S1047951121003310_ref2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(12)61023-X"},{"key":"S1047951121003310_ref8","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33518"},{"key":"S1047951121003310_ref13","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-008-0670-9"},{"key":"S1047951121003310_ref23","doi-asserted-by":"publisher","DOI":"10.1038\/ng1939"},{"key":"S1047951121003310_ref11","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-2-4"},{"key":"S1047951121003310_ref14","doi-asserted-by":"publisher","DOI":"10.1159\/000350686"},{"key":"S1047951121003310_ref3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1754.2010.01970.x"},{"key":"S1047951121003310_ref26","doi-asserted-by":"publisher","DOI":"10.1089\/gtmb.2009.0192"},{"key":"S1047951121003310_ref10","volume-title":"GeneReviews((R))","author":"Allanson","year":"1993"},{"key":"S1047951121003310_ref20","volume-title":"How to Read Pediatric ECGs","author":"Park","year":"2006"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951121003310","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,15]],"date-time":"2022-06-15T10:16:11Z","timestamp":1655288171000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951121003310\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,12]]},"references-count":39,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2022,6]]}},"alternative-id":["S1047951121003310"],"URL":"https:\/\/doi.org\/10.1017\/s1047951121003310","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"value":"1047-9511","type":"print"},{"value":"1467-1107","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,12]]},"assertion":[{"value":"\u00a9 The Author(s), 2021. Published by Cambridge University Press","name":"copyright","label":"Copyright","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This is an Open Access article, distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives licence (https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is unaltered and is properly cited. The written permission of Cambridge University Press must be obtained for commercial re-use or in order to create a derivative work.","name":"license","label":"License","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}