{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T17:39:15Z","timestamp":1769881155134,"version":"3.49.0"},"reference-count":16,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2021,6,27]],"date-time":"2021-06-27T00:00:00Z","timestamp":1624752000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100009192","name":"Alberta Innovates","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009192","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["obgyn.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Prenatal Diagnosis"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To document the clinical spectrum and outcomes of fetal double outlet right ventricle (DORV) without heterotaxy in a recent diagnostic era.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Prenatal cases of DORV consecutively diagnosed from 2007 to 2018 were retrospectively identified. Clinical records, including details regarding genetic testing and pre and postnatal imaging were reviewed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>DORV was diagnosed in 99 fetuses without heterotaxy. The most common anatomic subtype was subaortic ventricular septal defect (VSD) and normally related great arteries with (<jats:italic>n<\/jats:italic>\u00a0=\u00a045, 45%) or without (<jats:italic>n<\/jats:italic>\u00a0=\u00a013, 13%) pulmonary stenosis. The remainder had a subpulmonic VSD with transposed great arteries (<jats:italic>n<\/jats:italic>\u00a0=\u00a015, 15%), atrioventricular valve atresia (<jats:italic>n<\/jats:italic>\u00a0=\u00a024, 24%), or remote VSD (<jats:italic>n<\/jats:italic>\u00a0=\u00a02, 2%). A genetic diagnosis was found in 32 (34%) of 93 tested. Major extracardiac anomalies were found in 40 (40%), including 17\/24 (71%) with and 22\/69 (32%) without an abnormal karyotype, with VACTERL association in 9. Genetic and\/or extracardiac pathology was identified in 37\/58 (64%) with a subaortic VSD, 5\/15 (33%) with a subpulmonic VSD, 9\/24 (38%) of those with AV valve atresia and 2\/2 (100%) with a remote VSD. A genetic abnormality was a significant predictor of fetal demise (9\/37 vs 1\/62 <jats:italic>p<\/jats:italic>\u00a0&lt;\u00a00.01) or pregnancy termination (12\/35 vs 9\/64 <jats:italic>p<\/jats:italic>\u00a0=\u00a00.03).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Fetal DORV is associated with a high rate of genetic abnormalities and extracardiac pathology. The presence of genetic abnormalities impacts prenatal outcomes and parental decision\u2010making.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pd.5979","type":"journal-article","created":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T21:13:20Z","timestamp":1623532400000},"page":"1118-1126","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes"],"prefix":"10.1002","volume":"41","author":[{"given":"Aisling A.","family":"Young","sequence":"first","affiliation":[{"name":"Fetal\u00a0&amp; Neonatal Cardiology Program, Division of Pediatric Cardiology, Department of Pediatrics University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela","family":"McBrien","sequence":"additional","affiliation":[{"name":"Fetal\u00a0&amp; Neonatal Cardiology Program, Division of Pediatric Cardiology, Department of Pediatrics University of Alberta  Edmonton Alberta Canada"},{"name":"Women's and Children's Health Research Institute and Cardiovascular Research Institute University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oana","family":"Caluseriu","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kim","family":"Haberer","sequence":"additional","affiliation":[{"name":"Fetal\u00a0&amp; Neonatal Cardiology Program, Division of Pediatric Cardiology, Department of Pediatrics University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gayathri","family":"Wewala","sequence":"additional","affiliation":[{"name":"University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luke","family":"Eckersley","sequence":"additional","affiliation":[{"name":"Fetal\u00a0&amp; Neonatal Cardiology Program, Division of Pediatric Cardiology, Department of Pediatrics University of Alberta  Edmonton Alberta Canada"},{"name":"Women's and Children's Health Research Institute and Cardiovascular Research Institute University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2285-3202","authenticated-orcid":false,"given":"Lisa K.","family":"Hornberger","sequence":"additional","affiliation":[{"name":"Fetal\u00a0&amp; Neonatal Cardiology Program, Division of Pediatric Cardiology, Department of Pediatrics University of Alberta  Edmonton Alberta Canada"},{"name":"Women's and Children's Health Research Institute and Cardiovascular Research Institute University of Alberta  Edmonton Alberta Canada"},{"name":"Department of Obstetrics and Gynecology University of Alberta  Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,6,27]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.43.3.323"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.12170"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2010.07.009"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejogrb.2009.04.032"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1747-0803.2007.00069.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2010.206623"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1515\/jpm-2018-0316"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0028-1083840"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/pd.1500"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-013-0694-4"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.3109\/14767058.2011.561387"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2006.04.018"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.552364"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/uog.6309"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2015-103554"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320590116"}],"container-title":["Prenatal Diagnosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pd.5979","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pd.5979","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pd.5979","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T08:39:30Z","timestamp":1693298370000},"score":1,"resource":{"primary":{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/10.1002\/pd.5979"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,27]]},"references-count":16,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2021,8]]}},"alternative-id":["10.1002\/pd.5979"],"URL":"https:\/\/doi.org\/10.1002\/pd.5979","archive":["Portico"],"relation":{},"ISSN":["0197-3851","1097-0223"],"issn-type":[{"value":"0197-3851","type":"print"},{"value":"1097-0223","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,27]]},"assertion":[{"value":"2021-12-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-04-05","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-06-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}