{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:35:49Z","timestamp":1787016949245,"version":"build-2736575974"},"reference-count":16,"publisher":"BMJ","issue":"9","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Arch Dis Child"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Forecasting the prognosis of a child when diagnosed with Ebstein\u2019s anomaly is difficult. We, therefore, studied which factors at the time of diagnosis are associated with death during childhood.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>All consecutive patients (0\u201318 years) diagnosed with Ebstein\u2019s anomaly in the Netherlands between 1980 and 2014 were included. Survival curves were obtained using the Kaplan-Meier method. By using the Cox proportional hazard model, we analysed the factors (at diagnosis) that were associated with death.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We included 176 patients. Thirty-one patients (18%) died before the age of 18 years. The 1-year survival was 84% and remained stable at 82% from 35 months after diagnosis and onwards. Modified Ross Heart Failure Class 4 at the time of diagnosis was the most important risk factor for death during childhood (HR 12.5, 95%\u2009CI 4.4 to 35.9). Furthermore, diagnosis in the neonatal period (HR 4.2, 95%\u2009CI 1.5 to 12.0), severe tricuspid valve regurgitation (HR 2.4, 95%\u2009CI 1.2 to 5.0), severe right ventricular outflow tract obstruction (HR 3.7, 95%\u2009CI 1.8 to 7.7) and a patent ductus arteriosus (HR 2.8, 95%\u2009CI 1.3 to 6.0) at the time of diagnosis were univariately associated with death. Multivariable analysis showed that presentation with Heart Failure Class 4 and a ventricular septal defect is the strongest predictor of death in childhood and adolescence.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Patients with Ebstein\u2019s anomaly presenting with Heart Failure Class 4 and a ventricular septal defect have a high risk of death during childhood.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/archdischild-2017-313482","type":"journal-article","created":{"date-parts":[[2018,3,22]],"date-time":"2018-03-22T12:16:48Z","timestamp":1521721008000},"page":"859-863","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":12,"title":["Paediatric Ebstein\u2019s anomaly: how clinical presentation predicts mortality"],"prefix":"10.1136","volume":"103","author":[{"given":"Lianne M","family":"Geerdink","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tammo","family":"Delhaas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willem A","family":"Helbing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gideon J","family":"du Marchie Sarvaas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henriette ter","family":"Heide","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lieke","family":"Rozendaal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris L","family":"de Korte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petronella G M","family":"Peer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene M","family":"Kuipers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livia","family":"Kapusta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2018,3,22]]},"reference":[{"key":"2020050712450749000_103.9.859.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(99)70480-0"},{"key":"2020050712450749000_103.9.859.2","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1017\/S1047951113001169","article-title":"Dealing with Ebstein\u2019s anomaly","volume":"24","author":"Geerdink","year":"2014","journal-title":"Cardiol 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adult in 52 patients","volume":"14","author":"Flores Arizmendi","year":"2004","journal-title":"Cardiol Young"},{"key":"2020050712450749000_103.9.859.11","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1161\/01.CIR.68.3.534","article-title":"Two-dimensional echocardiographic--surgical correlation in Ebstein\u2019s anomaly: preoperative determination of patients requiring tricuspid valve plication vs replacement","volume":"68","author":"Shiina","year":"1983","journal-title":"Circulation"},{"key":"2020050712450749000_103.9.859.12","doi-asserted-by":"publisher","DOI":"10.1007\/BF00798207"},{"key":"2020050712450749000_103.9.859.13","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.108.820217"},{"key":"2020050712450749000_103.9.859.14","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1111\/chd.12404","article-title":"Surgical outcome in pediatric patients with Ebstein\u2019s anomaly: a multicenter, long-term study","volume":"12","author":"Geerdink","year":"2017","journal-title":"Congenit Heart Dis"},{"key":"2020050712450749000_103.9.859.15","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.015839"},{"key":"2020050712450749000_103.9.859.16","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1016\/S0022-5223(19)35302-4","article-title":"A new reconstructive operation for Ebstein\u2019s anomaly of the tricuspid valve","volume":"96","author":"Carpentier","year":"1988","journal-title":"J Thorac Cardiovasc Surg"}],"container-title":["Archives of Disease in 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