{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T14:19:58Z","timestamp":1778768398966,"version":"3.51.4"},"reference-count":33,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T00:00:00Z","timestamp":1777507200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>Scimitar syndrome is a rare congenital cardiopulmonary anomaly with marked anatomical and clinical heterogeneity, and long-term outcome data remain limited. We reviewed our single-center experience over a 33-year period. Patients evaluated between 1992 and 2025 were retrospectively analyzed. A total of 104 patients were included, with female predominance (63, 60.6%). The median age at first presentation was 0.4 years (IQR 0.0\u201316.7; range 1 day\u201368 years) with 59 patients (56.7%) presenting during infancy. At last follow-up, the median age was 18.5 years (IQR 8.7\u201330.6; range 60 days\u201370 years), with a median follow-up duration of 9.5 years (IQR 3.7\u201316.1). Dextrocardia was observed in 76 patients (73.1%). The most common associated defect was atrial septal defect (37 patients, 35.6%), while 23 patients (22.1%) had no additional cardiac defects. Respiratory manifestations predominated at presentation and follow-up. However, 18 patients (17.3%) were asymptomatic at diagnosis and 44 (42.3%) at follow-up. Partial anomalous pulmonary venous drainage was present in 87 patients (83.6%), and aorto-pulmonary collaterals in 70 (67.3%). Cardiac catheterization was performed in 78 patients (75.0%), and 47 (45.2%) underwent surgery. At last documented follow-up, 101 of 104 patients (97.1%) were alive, with three deaths occurring during follow-up.<\/jats:p>","DOI":"10.3390\/jcdd13050196","type":"journal-article","created":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T06:50:01Z","timestamp":1777618201000},"page":"196","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Long-Term Outcomes and Imaging Characteristics of Patients with Scimitar Syndrome"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6904-535X","authenticated-orcid":false,"given":"Hicran G\u00fcl","family":"Emral","sequence":"first","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-2151-6145","authenticated-orcid":false,"given":"Thao V. N.","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilaria","family":"Bo","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3740-6429","authenticated-orcid":false,"given":"Thomas","family":"Semple","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London, London SW7 2AZ, UK"},{"name":"Radiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5698-5828","authenticated-orcid":false,"given":"Julene S.","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"},{"name":"Cardiovascular and Genomics Research Institute, City St. George\u2019s, University of London, London EC1V 0HB, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7573-8235","authenticated-orcid":false,"given":"Piers E. F.","family":"Daubeney","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"},{"name":"National Heart and Lung Institute, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3301-8173","authenticated-orcid":false,"given":"Michael L.","family":"Rigby","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylvia","family":"Krupickova","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Department, Royal Brompton Hospital, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SW3 6NP, UK"},{"name":"National Heart and Lung Institute, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1016\/0002-9149(92)91198-D","article-title":"The adult form of the scimitar syndrome","volume":"70","author":"Dupuis","year":"1992","journal-title":"Am. 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Heart Surg."}],"container-title":["Journal of Cardiovascular Development and Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2308-3425\/13\/5\/196\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T13:48:04Z","timestamp":1778766484000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2308-3425\/13\/5\/196"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,30]]},"references-count":33,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2026,5]]}},"alternative-id":["jcdd13050196"],"URL":"https:\/\/doi.org\/10.3390\/jcdd13050196","relation":{},"ISSN":["2308-3425"],"issn-type":[{"value":"2308-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,30]]}}}