{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T17:06:17Z","timestamp":1784567177652,"version":"3.55.0"},"reference-count":9,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T00:00:00Z","timestamp":1783555200000},"content-version":"vor","delay-in-days":8,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>PHACE syndrome is a neurocutaneous syndrome characterized by posterior fossa malformations, haemangiomas (primarily on the face, scalp, and neck), arterial anomalies, cardiac abnormalities\/coarctation of the aorta, and eye abnormalities. Cardiac abnormalities associated with PHACE syndrome typically present with coarctation of the aorta. Cases presenting with severe aortic arch tortuosity are rare.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>The patient was delivered by caesarean section at 37 weeks and 0 days of gestation with a weight of 2546\u2005g. She was diagnosed with a right aortic arch, aberrant left subclavian artery and left ductus arteriosus during the foetal period and was admitted to the neonatal intensive care unit for further evaluation. Echocardiography raised suspicion of aortic arch hypoplasia. A contrast-enhanced computed tomography showed severe aortic arch tortuosity. Cardiac catheterization performed at 3 months of age showed extremely elevated left ventricular pressure. An extra-anatomical bypass was performed at 4 months of age. A diagnosis of PHACE syndrome was made based on the combination of specific aortic arch anomalies, cerebral carotid artery anomalies, and haemangioma in the deep neck space.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>In this case, severe aortic arch tortuosity detected immediately after birth resulted in stenosis. If this distinctive aortic morphology is present, PHACE syndrome should be considered in the differential diagnosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytag486","type":"journal-article","created":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T11:54:33Z","timestamp":1783511673000},"source":"Crossref","is-referenced-by-count":0,"title":["PHACE syndrome with severe aortic arch tortuosity: a case report"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-2029-9962","authenticated-orcid":false,"given":"Yasuyuki","family":"Masuda","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Kurashiki Central Hospital , 1-1-1, Miwa, Kurashiki, Okayama 710-8602 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kayo","family":"Ogino","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kurashiki Central Hospital , 1-1-1, Miwa, Kurashiki, Okayama 710-8602 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiro","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kurashiki Central Hospital , 1-1-1, Miwa, Kurashiki, Okayama 710-8602 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenji","family":"Waki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kurashiki Central Hospital , 1-1-1, Miwa, Kurashiki, Okayama 710-8602 ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,7,9]]},"reference":[{"key":"2026072012474149700_ytag486-B1","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1001\/archderm.1996.03890270083012","article-title":"PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities","volume":"132","author":"Frieden","year":"1996","journal-title":"Arch Dermatol"},{"key":"2026072012474149700_ytag486-B2","doi-asserted-by":"crossref","DOI":"10.1016\/j.spen.2024.101152","article-title":"PHACE syndrome: a review","volume":"51","author":"Keith","year":"2024","journal-title":"Semin Pediatr Neurol"},{"key":"2026072012474149700_ytag486-B3","doi-asserted-by":"crossref","first-page":"333","DOI":"10.4103\/apc.APC_188_18","article-title":"Corkscrew aortic arch in PHACES syndrome: multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome","volume":"12","author":"Sheth","year":"2019","journal-title":"Ann Pediatr Cardiol"},{"key":"2026072012474149700_ytag486-B4","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.jpeds.2016.07.054","article-title":"PHACE syndrome: consensus-derived diagnosis and care recommendations","volume":"178","author":"Garzon","year":"2016","journal-title":"J Pediatr"},{"key":"2026072012474149700_ytag486-B5","doi-asserted-by":"crossref","first-page":"1948","DOI":"10.1016\/j.amjcard.2013.08.025","article-title":"Congenital cardiac, aortic arch, and vascular bed anomalies in PHACE syndrome (from the International PHACE Syndrome Registry)","volume":"112","author":"Bayer","year":"2013","journal-title":"Am J Cardiol"},{"key":"2026072012474149700_ytag486-B6","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/S0003-4975(02)03554-3","article-title":"Repair of a complex aortic arch anomaly associated with cutaneous hemangioma","volume":"74","author":"Gargiulo","year":"2002","journal-title":"Ann Thorac Surg"},{"key":"2026072012474149700_ytag486-B7","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1542\/peds.113.2.412","article-title":"Ipsilateral hemangioma and aortic arch anomalies in posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac defects and eye abnormalities (PHACE) anomaly: report and review","volume":"113","author":"Bronzetti","year":"2004","journal-title":"Pediatrics"},{"key":"2026072012474149700_ytag486-B8","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1016\/S0300-8932(10)70306-7","article-title":"Complex aortic coarctation and PHACE syndrome","volume":"63","author":"Prada","year":"2010","journal-title":"Rev Esp Cardiol"},{"key":"2026072012474149700_ytag486-B9","doi-asserted-by":"crossref","first-page":"507","DOI":"10.2350\/12-05-1203-CR.1","article-title":"Postmortem vascular pathology in PHACES syndrome: a case report","volume":"15","author":"Chad","year":"2012","journal-title":"Pediatr Dev Pathol"}],"container-title":["European Heart Journal - Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ehjcr\/advance-article-pdf\/doi\/10.1093\/ehjcr\/ytag486\/68740925\/ytag486.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjcr\/article-pdf\/10\/7\/ytag486\/68740925\/ytag486.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjcr\/article-pdf\/10\/7\/ytag486\/68740925\/ytag486.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T16:47:50Z","timestamp":1784566070000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcr\/article\/doi\/10.1093\/ehjcr\/ytag486\/8729329"}},"subtitle":[],"editor":[{"given":"Andriana","family":"Anagnostopoulou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Alessandro","family":"Palmieri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Deborah","family":"Cosmi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Deepti","family":"Ranganathan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":9,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7,2]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjcr\/ytag486","relation":{},"ISSN":["2514-2119"],"issn-type":[{"value":"2514-2119","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,7]]},"published":{"date-parts":[[2026,7]]},"article-number":"ytag486"}}