{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T01:51:25Z","timestamp":1760233885376,"version":"build-2065373602"},"reference-count":16,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2021,3,3]],"date-time":"2021-03-03T00:00:00Z","timestamp":1614729600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Uhl\u2019s anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl\u2019s anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.<\/jats:p>","DOI":"10.3390\/children8030190","type":"journal-article","created":{"date-parts":[[2021,3,3]],"date-time":"2021-03-03T20:33:57Z","timestamp":1614803637000},"page":"190","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Case of Prenatally Diagnosed Uhl\u2019s Anomaly with Absent Pulmonary Valve Leaflets and Dysplastic Tricuspid Valve"],"prefix":"10.3390","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2411-2309","authenticated-orcid":false,"given":"Taehong","family":"Kim","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5834-0900","authenticated-orcid":false,"given":"Hoon","family":"Ko","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joung-Hee","family":"Byun","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyoung Doo","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3972-456X","authenticated-orcid":false,"given":"Hyungtae","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kwangho","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1138-433X","authenticated-orcid":false,"given":"Joo-Young","family":"Na","sequence":"additional","affiliation":[{"name":"Department of Pathology, Pusan National University Yangsan Hospital, Yangsan-si 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,3,3]]},"reference":[{"key":"ref_1","first-page":"197","article-title":"A previously undescribed congenital malformation of the heart: Almost total absence of the myocardium of the right ventricle","volume":"91","author":"Uhl","year":"1952","journal-title":"Bull. Johns Hopkins Hosp."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1483","DOI":"10.1161\/01.CIR.93.8.1483","article-title":"Uhl\u2019s anomaly revisited","volume":"93","author":"Uhl","year":"1996","journal-title":"Circulation"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.ijcard.2016.02.048","article-title":"Uhl\u2019s anomaly: Clinical spectrum and pathophysiology","volume":"209","author":"Blom","year":"2016","journal-title":"Int. J. Cardiol."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Osler, W.M. (1905). The Principles and Practice of Medicine, D. Appleton. [6th ed.].","DOI":"10.2307\/3403146"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1136\/hrt.69.2.142","article-title":"Dysplastic conditions of the right ventricular myocardium: Uhl\u2019s anomaly vs arrhythmogenic right ventricular dysplasia","volume":"69","author":"Gerlis","year":"1993","journal-title":"Br. 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Electrocardiogram, Echocardiography, and Magnetic resonance imaging characteristics in Uhl\u2019s disease","volume":"115","author":"Otmani","year":"2007","journal-title":"Circulation"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"90","DOI":"10.4103\/2211-4122.166086","article-title":"Uhl\u2019s anomaly with absent tricuspid valve in an infant","volume":"25","author":"Taksande","year":"2015","journal-title":"J. Cardiovasc. Echogr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1017\/S1047951102000410","article-title":"Successful surgical repair of Uhl\u2019s anomaly","volume":"12","author":"Azhari","year":"2002","journal-title":"Cardiol. Young"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1067\/mtc.2001.116319","article-title":"A case of Uhl anomaly treated with one and a half ventricle repair combined with partial right ventriculectomy in infancy","volume":"122","author":"Yoshii","year":"2001","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1590\/S0066-782X2001000700008","article-title":"Uhl\u2019s anomaly. Differential diagnosis and indications for cardiac transplantation in an infant","volume":"77","author":"Ikari","year":"2001","journal-title":"Arq. Bras. 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