{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T07:59:57Z","timestamp":1761897597619,"version":"build-2065373602"},"reference-count":24,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2021,8,25]],"date-time":"2021-08-25T00:00:00Z","timestamp":1629849600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>(1) Secundum type atrial septal defect (ASD II) is usually considered a relatively benign cardiac lesion amenable to elective closure at preschool age. Patients with trisomy 21 (T21), however, are known to have a higher susceptibility for pulmonary vascular disease (PVD). Therefore, T21 children may present with clinical symptoms earlier than those without associated anomalies. In addition, early PVD may even preclude closure in selected T21 patients. (2) We performed a retrospective analysis of the German National Register for Congenital Heart Defects including T21 patients with associated isolated ASD II. We report incidence, demographics, therapeutic strategy, outcome, and survival of this cohort. (3) Of 46,628 patients included in the registry, 1549 (3.3%) had T21. Of these, 156 (49.4% female) had an isolated ASD II. Fifty-four patients (34.6%) underwent closure at 6.4 \u00b1 9.9 years of age. Over a cumulative follow-up (FU) of 1148 patient-years, (median 7.4 years), only one patient developed Eisenmenger syndrome and five patients died. Survival of T21 patients without PVD was not statistically different to age- and gender-matched controls from the normal population (p = 0.62), whereas children with uncorrected T21\/ASD II (including patients with severe PVD, in whom ASD-closure was considered contraindicated) showed a significantly higher mortality. (4) The outcome of T21-patients with ASD II and without PVD is excellent. However, PVD, either precluding ASD-closure or development of progressive PVD after ASD-closure, is associated with significant mortality in this cohort. Thus T21 patients with ASD II who fulfill general criteria for closure and without PVD should be offered defect closure analogous to patients without T21.<\/jats:p>","DOI":"10.3390\/jcm10173807","type":"journal-article","created":{"date-parts":[[2021,8,26]],"date-time":"2021-08-26T00:19:26Z","timestamp":1629937166000},"page":"3807","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Secundum Type Atrial Septal Defect in Patients with Trisomy 21\u2014Therapeutic Strategies, Outcome, and Survival: A Nationwide Study of the German National Registry for Congenital Heart Defects"],"prefix":"10.3390","volume":"10","author":[{"given":"Astrid E.","family":"Lammers","sequence":"first","affiliation":[{"name":"Department of Cardiology III\u2014Adult Congenital and Valvular Heart Disease, University Hospital Muenster, 48149 M\u00fcnster, Germany"},{"name":"Department of Paediatric Cardiology, University Hospital Muenster, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Stegger","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, University Hospital Muenster, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc-Andr\u00e9","family":"Koerten","sequence":"additional","affiliation":[{"name":"Department of Cardiology III\u2014Adult Congenital and Valvular Heart Disease, University Hospital Muenster, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul C.","family":"Helm","sequence":"additional","affiliation":[{"name":"National Register for Congenital Heart Defects, 13353 Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ulrike M.","family":"Bauer","sequence":"additional","affiliation":[{"name":"National Register for Congenital Heart Defects, 13353 Berlin, Germany"},{"name":"DZHK (German Centre for Cardiovascular Research), 10785 Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut","family":"Baumgartner","sequence":"additional","affiliation":[{"name":"Department of Cardiology III\u2014Adult Congenital and Valvular Heart Disease, University Hospital Muenster, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anselm S.","family":"Uebing","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Angeborene Herzfehler und Kinderkardiologie, Universit\u00e4tsklinikum Schleswig-Holstein, 24105 Kiel, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s00431-017-3041-6","article-title":"Dynamics in prevalence of Down syndrome in children with congenital heart disease","volume":"177","author":"Pfitzer","year":"2018","journal-title":"Eur. 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