{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T08:01:51Z","timestamp":1770883311577,"version":"3.50.1"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T00:00:00Z","timestamp":1745712000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T00:00:00Z","timestamp":1745712000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003793","name":"Hj\u00e4rt-Lungfonden","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003793","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009780","name":"Region Sk\u00e5ne","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011077","name":"Sk\u00e5nes universitetssjukhus","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100011077","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004359","name":"Vetenskapsr\u00e5det","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003252","name":"Lund University","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003252","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Cardiol"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The \u201cnutmeg lung pattern\u201d on fetal magnetic resonance imaging (MRI) indicates pulmonary lymphangiectasia. This is associated with adverse outcomes, particularly in fetuses with congenital heart defects and impaired pulmonary venous return. Whereas lymphedema is common in fetuses with Turner syndrome, pulmonary lymphangiectasia is not. A 26-year-old woman presented with a fetus with hypoplastic left heart syndrome (HLHS) without restrictive atrial septum (RAS). The family declined amniocentesis, yet non-invasive prenatal testing showed an increased risk for Turner syndrome. The patient underwent a fetal MRI as part of a blinded research protocol. Postnatal echocardiogram confirmed the fetal echocardiographic findings without evidence of RAS. Norwood stage I palliation was performed at two days of age. Significant neonatal respiratory morbidity including pneumonia, diaphragmatic and vocal cord pareses and chylothorax occurred. Subsequent review of fetal MRI revealed a prominent thoracic duct and mild pulmonary lymphangiectasia. Turner syndrome was confirmed by genetic testing. After one month, the patient was discharged to her home hospital with respiratory support, which was discontinued a few weeks later. Respiratory problems continued, but the vocal cord paresis resolved over time. This is a unique case of a fetus with HLHS\/non-RAS with mild fetal pulmonary lymphangiectasia, and significant neonatal respiratory morbidity, probably in part due to Turner syndrome. The infant survived the neonatal period and underwent uneventful Glenn surgery. The patient\u2019s tolerance to the total cavopulmonary connection (TCPC) procedure is yet to be seen. An MRI lymphography should precede it to assess residual lymphatic abnormalities and serve as baseline for post-TCPC changes.<\/jats:p>","DOI":"10.1007\/s00246-025-03873-x","type":"journal-article","created":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T02:13:43Z","timestamp":1745720023000},"page":"1383-1388","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Nutmeg Lung Pattern in a Fetus with Hypoplastic Left Heart Syndrome and Turner Syndrome"],"prefix":"10.1007","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0879-6499","authenticated-orcid":false,"given":"Katrin","family":"Fricke","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katarina","family":"\u00d6vermo Tyd\u00e9n","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gunnar","family":"Bergman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Hedstr\u00f6m","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,4,27]]},"reference":[{"issue":"10","key":"3873_CR1","doi-asserted-by":"publisher","first-page":"1237","DOI":"10.1007\/s00247-014-2994-0","volume":"44","author":"T Victoria","year":"2014","unstructured":"Victoria T, Andronikou S (2014) The fetal MR appearance of \u201cnutmeg lung\u201d: findings in 8 cases linked to pulmonary lymphangiectasia. Pediatr Radiol 44(10):1237\u20131242","journal-title":"Pediatr Radiol"},{"issue":"4","key":"3873_CR2","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1007\/s00247-015-3514-6","volume":"46","author":"D Saul","year":"2016","unstructured":"Saul D et al (2016) Hypoplastic left heart syndrome and the nutmeg lung pattern in utero: a cause and effect relationship or prognostic indicator? Pediatr Radiol 46(4):483\u2013489","journal-title":"Pediatr Radiol"},{"issue":"10","key":"3873_CR3","doi-asserted-by":"publisher","first-page":"1809","DOI":"10.1007\/s00247-021-05061-4","volume":"51","author":"CA Barrera","year":"2021","unstructured":"Barrera CA et al (2021) Prognostic value of the nutmeg lung pattern\/lymphangiectasia on fetal magnetic resonance imaging. 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Respiration 101(1):84\u201396","journal-title":"Respiration"},{"key":"3873_CR6","doi-asserted-by":"publisher","first-page":"1058567","DOI":"10.3389\/fped.2023.1058567","volume":"11","author":"C Bauer","year":"2023","unstructured":"Bauer C et al (2023) Current diagnostic and therapeutic strategies for the management of lymphatic insufficiency in patients with hypoplastic left heart syndrome. Front Pediatr 11:1058567","journal-title":"Front Pediatr"},{"issue":"1","key":"3873_CR7","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1210\/endrev\/bnac016","volume":"44","author":"CH Gravholt","year":"2023","unstructured":"Gravholt CH et al (2023) The changing face of turner syndrome. 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