{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T17:46:58Z","timestamp":1770140818066,"version":"3.49.0"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T00:00:00Z","timestamp":1712880000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:p>Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks\u2009+\u20096 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (&amp;gt;85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.<\/jats:p>","DOI":"10.3389\/fcvm.2024.1351530","type":"journal-article","created":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T04:25:45Z","timestamp":1712895945000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome"],"prefix":"10.3389","volume":"11","author":[{"given":"M.","family":"Masci","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Missineo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. M.","family":"Campanale","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Moras","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. C.","family":"Colucci","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Pasquini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Toscano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,4,12]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1177\/21501351221122972","article-title":"Isolated vascular rings are common cardiovascular malformations","volume":"14","author":"Evans","year":"2023","journal-title":"World J Pediatr Congenit Heart Surg"},{"key":"B2","doi-asserted-by":"publisher","first-page":"2985","DOI":"10.1080\/14767058.2019.1676413","article-title":"Prenatal incidence of isolated right aortic arch and double aortic arch","volume":"34","author":"Vigneswaran","year":"2021","journal-title":"J Matern Fetal Neonatal Med"},{"key":"B3","doi-asserted-by":"publisher","first-page":"151128","DOI":"10.1016\/j.sempedsurg.2021.151128","article-title":"Vascular rings","volume":"30","author":"Worhunsky","year":"2021","journal-title":"Semin Pediatr Surg"},{"key":"B4","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1111\/j.1540-8175.2007.00554.x","article-title":"Double aortic arch and D-transposition of the great arteries","volume":"25","author":"Cui","year":"2008","journal-title":"Echocardiography"},{"key":"B5","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1016\/0002-8703(94)90143-0","article-title":"Double aortic arch and bilateral patent ducti arteriosi associated with transposition of the great arteries: missing clinical link in an embryologic theory","volume":"127","author":"Shirali","year":"1994","journal-title":"Am Heart J"},{"key":"B6","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1007\/BF02807235","article-title":"Double aortic arch in D-transposition of the great arteries complicated by tracheobronchomalacia","volume":"18","author":"T\u016fma","year":"1995","journal-title":"Cardiovasc Intervent Radiol"},{"key":"B7","first-page":"398","article-title":"Double aortic arch in D-transposition of the great arteries: confirmation of dominant arch by magnetic resonance imaging","volume":"27","author":"McMahon","year":"2000","journal-title":"Tex Heart Inst J"},{"key":"B8","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1136\/hrt.30.4.579","article-title":"Double aortic arch associated with complete transposition of the great vessels","volume":"30","author":"Higashino","year":"1968","journal-title":"Br Heart J"},{"key":"B9","doi-asserted-by":"publisher","first-page":"1701","DOI":"10.7863\/ultra.15.14.10012","article-title":"Fetal diagnosis of D-transposition of the great arteries associated with a double aortic arch","volume":"34","author":"Anuwutnavin","year":"2015","journal-title":"J Ultrasound Med"},{"key":"B10","doi-asserted-by":"publisher","first-page":"382","DOI":"10.1002\/pd.2472","article-title":"Prenatal diagnosis of double aortic arch and D-transposition of the great arteries: a case report","volume":"30","author":"Yao","year":"2010","journal-title":"Prenat Diagn"},{"key":"B11","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1016\/j.ijporl.2006.08.025","article-title":"The presentation and management of vascular rings: an otolaryngology perspective","volume":"71","author":"Shah","year":"2007","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"B12","first-page":"294","article-title":"Th. Aortenbogen, Ringformiger, bei einemneugenbornen, blausuchtigen Kinde","volume":"16","author":"Von Siebold","year":"1837","journal-title":"J Geburtschulfe Frauenzimmer Kinderkraukheiten"},{"key":"B13","doi-asserted-by":"publisher","first-page":"570","DOI":"10.1016\/0003-4975(93)90905-w","article-title":"Repair of double aortic arch associated with D-transposition of the great arteries","volume":"56","author":"Kupferschmid","year":"1993","journal-title":"Ann Thorac Surg"},{"key":"B14","doi-asserted-by":"publisher","first-page":"e76","DOI":"10.1016\/j.hlc.2017.03.155","article-title":"D-TGA combined with left arch atresia of a double aortic arch","volume":"26","author":"Zhou","year":"2017","journal-title":"Heart Lung Circ"},{"key":"B15","doi-asserted-by":"publisher","first-page":"564","DOI":"10.1136\/archdischild-2020-318946","article-title":"Postnatal impact of a prenatally diagnosed double aortic arch","volume":"106","author":"Vigneswaran","year":"2021","journal-title":"Arch Dis Child"},{"key":"B16","doi-asserted-by":"publisher","first-page":"2114","DOI":"10.1016\/S0735-1097(01)01286-4","article-title":"Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching","volume":"37","author":"McElhinney","year":"2001","journal-title":"J Am Coll Cardiol"},{"key":"B17","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1086\/339079","article-title":"CFC1 mutations in patients with transposition of the great arteries and double-outlet right ventricle","volume":"70","author":"Goldmuntz","year":"2002","journal-title":"Am J Hum Genet"},{"key":"B18","doi-asserted-by":"publisher","first-page":"792","DOI":"10.1002\/ajmg.a.35849","article-title":"The phenotypic spectrum of ZIC3 mutations includes isolated d-transposition of the great arteries and double outlet right ventricle","volume":"161","author":"D\u2019Alessandro","year":"2013","journal-title":"Am J Med Genet A"},{"key":"B19","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1002\/humu.20480","article-title":"Elucidation of penetrance variability of a ZIC3 mutation in a family with complex heart defects and functional analysis of ZIC3 mutations in the first zinc finger domain","volume":"28","author":"Chhin","year":"2007","journal-title":"Hum Mutat"},{"key":"B20","doi-asserted-by":"publisher","first-page":"430","DOI":"10.1007\/s00246-002-0385-z","article-title":"Vascular rings and their effect on tracheal geometry","volume":"24","author":"Fleenor","year":"2003","journal-title":"Pediatr Cardiol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1186\/s13052-023-01430-x","article-title":"Long term respiratory morbidity in patients with vascular rings: a review","volume":"49","author":"Porcaro","year":"2023","journal-title":"Ital J Pediatr"},{"key":"B22","doi-asserted-by":"publisher","first-page":"1900382","DOI":"10.1183\/13993003.00382-2019","article-title":"ERS statement on tracheomalacia and bronchomalacia in children","volume":"54","author":"Wallis","year":"2019","journal-title":"Eur Respir J"},{"key":"B23","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1001\/archotol.1994.01880280077015","article-title":"Congenital tracheal stenosis. The otolaryngologist's perspective","volume":"120","author":"Hoffer","year":"1994","journal-title":"Arch Otolaryngol Head Neck Surg"}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2024.1351530\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T04:25:49Z","timestamp":1712895949000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2024.1351530\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,12]]},"references-count":23,"alternative-id":["10.3389\/fcvm.2024.1351530"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2024.1351530","relation":{},"ISSN":["2297-055X"],"issn-type":[{"value":"2297-055X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,12]]},"article-number":"1351530"}}