{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T20:19:36Z","timestamp":1785356376671,"version":"3.55.0"},"reference-count":16,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T00:00:00Z","timestamp":1679529600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>As yet, there is no agreed definition for the so-called \u201chypoplastic left heart syndrome\u201d. Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a \u201csyndrome\u201d in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described \u201chypoplasia of the aortic outflow tract complex\u201d. In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.<\/jats:p>","DOI":"10.3390\/jcdd10040133","type":"journal-article","created":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T02:35:26Z","timestamp":1679538926000},"page":"133","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["What Is the Hypoplastic Left Heart Syndrome?"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5163-9021","authenticated-orcid":false,"given":"Robert H.","family":"Anderson","sequence":"first","affiliation":[{"name":"Biosciences Division, Newcastle University, Newcastle upon Tyne NE1 7RU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrian","family":"Crucean","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiac Surgery, Birmingham Women\u2019s and Children\u2019s Hospital, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane E.","family":"Spicer","sequence":"additional","affiliation":[{"name":"Heart Institute, Johns Hopkins All Children\u2019s Hospital, St. Petersberg, FL 33701, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1017\/S1047951106000291","article-title":"The nomenclature, definition and classification of hypoplastic left heart syndrome","volume":"16","author":"Tchervenkov","year":"2006","journal-title":"Cardiol. Young"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1038\/ng.3870","article-title":"The complex genetics of hypoplastic left heart syndrome","volume":"49","author":"Liu","year":"2017","journal-title":"Nat. Gene"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1038\/s41569-020-00500-5","article-title":"Clarification of the definition of hypoplastic left heart syndrome","volume":"18","author":"Anderson","year":"2021","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1053\/j.semtcvs.2020.02.019","article-title":"Pathologic characteristics of 119 archived specimens showing the phenotypic features of hypoplastic left heart syndrome","volume":"32","author":"Stephens","year":"2020","journal-title":"Sem. Thorac. Cardiovasc. Surg."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.1016\/S0031-3955(16)30727-1","article-title":"The hypoplastic left heart syndrome: An analysis of 101 cases","volume":"5","author":"Noonan","year":"1958","journal-title":"Pediat. Clin. N. Am."},{"key":"ref_6","first-page":"61","article-title":"Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes","volume":"1","author":"Lev","year":"1952","journal-title":"Lab. Investig."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/S0025-7125(16)33218-7","article-title":"Some newer concepts of the pathology of congenital heart disease","volume":"50","author":"Lev","year":"1966","journal-title":"Med. Clin. N. Am."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1093\/ejcts\/ezaa188","article-title":"Guidelines for the management of neonates and infants with hypoplastic left heart syndrome: The European Association for Cardio-Thoracic Surgery (EACTS) and the Association for European Paediatric and Congenital Cardiology (AEPC) Hypoplastic Left Heart Syndrome Guidelines Task Force","volume":"58","author":"Alphonso","year":"2020","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1093\/oxfordjournals.eurheartj.a061505","article-title":"The anatomical heterogeneity of hearts lacking a patent communication between the left atrium and the ventricular mass (\u2018mitral atresia\u2019) in presence of a patent aortic valve","volume":"4","author":"Mickell","year":"1983","journal-title":"Eur. Heart J."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1056\/NEJM198301063080106","article-title":"Physiologic repair of aortic atresia\u2013hypoplastic left heart syndrome","volume":"308","author":"Norwood","year":"1983","journal-title":"N. Engl. J. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1136\/hrt.51.3.252","article-title":"Mitral atresia. Morphological details","volume":"51","author":"Wenink","year":"1984","journal-title":"Br. Heart J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1016\/S0003-4975(98)00803-0","article-title":"Biventricular repair in neonates with hypoplastic left heart complex","volume":"66","author":"Tchervenkov","year":"1998","journal-title":"Ann. Thor. Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1002\/dvdy.24521","article-title":"Endocardial fibroelastosis is secondary to hemodynamic alterations in the chick embryonic model of hypoplastic left heart syndrome","volume":"247","author":"Pesevski","year":"2018","journal-title":"Dev. Dyn."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/j.jtcvs.2019.08.101","article-title":"Flow disturbances and the development of endocardial fibroelastosis","volume":"159","author":"Weixler","year":"2020","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13023-017-0683-4","article-title":"Re-evaluation of hypoplastic left heart syndrome from a developmental and morphological perspective","volume":"12","author":"Crucean","year":"2017","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"dmm049077","DOI":"10.1242\/dmm.049077","article-title":"A mouse model of hypoplastic left heart syndrome demonstrating left heart hypoplasia and retrograde aortic arch flow","volume":"14","author":"Rahman","year":"2021","journal-title":"Dis. Models Mech."}],"container-title":["Journal of Cardiovascular Development and Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2308-3425\/10\/4\/133\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:01:01Z","timestamp":1760122861000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2308-3425\/10\/4\/133"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,23]]},"references-count":16,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2023,4]]}},"alternative-id":["jcdd10040133"],"URL":"https:\/\/doi.org\/10.3390\/jcdd10040133","relation":{},"ISSN":["2308-3425"],"issn-type":[{"value":"2308-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,23]]}}}