{"id":"https://openalex.org/W3123354792","doi":"https://doi.org/10.32604/chd.2021.014409","title":"Kabuki-Syndrome and Congenital Heart Disease\u2014A Twenty-Year Institutional Experience","display_name":"Kabuki-Syndrome and Congenital Heart Disease\u2014A Twenty-Year Institutional Experience","publication_year":2021,"publication_date":"2021-01-01","ids":{"openalex":"https://openalex.org/W3123354792","doi":"https://doi.org/10.32604/chd.2021.014409","mag":"3123354792"},"language":"en","primary_location":{"id":"doi:10.32604/chd.2021.014409","doi":"https://doi.org/10.32604/chd.2021.014409","is_oa":true,"landing_page_url":"https://doi.org/10.32604/chd.2021.014409","pdf_url":"https://www.techscience.com/ueditor/files/TSP_CHD-16-2/TSP_CHD_14409/TSP_CHD_14409.pdf","source":{"id":"https://openalex.org/S173391249","display_name":"Congenital Heart Disease","issn_l":"1747-079X","issn":["1747-079X","1747-0803"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","jufo-1","ki-jl-1","norway-1","oarl","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Congenital Heart Disease","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://www.techscience.com/ueditor/files/TSP_CHD-16-2/TSP_CHD_14409/TSP_CHD_14409.pdf","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5050958778","display_name":"Reghan L. Conrey","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Reghan Conrey","raw_affiliation_strings":["Baylor College of Medicine, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Baylor College of Medicine, Houston, USA","institution_ids":["https://openalex.org/I181547552"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5062202338","display_name":"Sebastian C. Tume","orcid":"https://orcid.org/0000-0002-8582-4759","observed_orcids":["https://orcid.org/0000-0002-8582-4759"]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sebastian Tume","raw_affiliation_strings":["Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, USA","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5051679299","display_name":"Carlos Bonilla\u2010Ram\u00edrez","orcid":"https://orcid.org/0000-0002-8730-7624","observed_orcids":["https://orcid.org/0000-0002-8730-7624"]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Carlos Bonilla-Ramirez","raw_affiliation_strings":["Division of Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, USA","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5033405102","display_name":"Seema R. Lalani","orcid":"https://orcid.org/0000-0003-0707-657X","observed_orcids":["https://orcid.org/0000-0003-0707-657X"]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Seema Lalani","raw_affiliation_strings":["Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, USA","institution_ids":["https://openalex.org/I181547552"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Dean McKenzie","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Dean McKenzie","raw_affiliation_strings":["Division of Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, USA","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5104362116","display_name":"Marc Marc","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Marc","raw_affiliation_strings":["Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, USA","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5060550245","display_name":"ers","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I181547552","display_name":"Baylor College of Medicine","ror":"https://ror.org/02pttbw34","country_code":"US","type":"education","lineage":["https://openalex.org/I181547552","https://openalex.org/I2801539370"]},{"id":"https://openalex.org/I4210147586","display_name":"Texas Children's Hospital","ror":"https://ror.org/05cz92x43","country_code":"US","type":"funder","lineage":["https://openalex.org/I4210147586"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"ers","raw_affiliation_strings":["Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, USA","institution_ids":["https://openalex.org/I181547552","https://openalex.org/I4210147586"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5050958778"],"corresponding_institution_ids":["https://openalex.org/I181547552"],"apc_list":{"value":3100,"currency":"USD","value_usd":3100},"apc_paid":{"value":3100,"currency":"USD","value_usd":3100},"fwci":0.1801,"has_fulltext":true,"cited_by_count":4,"citation_normalized_percentile":{"value":0.44251887,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":97},"biblio":{"volume":"16","issue":"2","first_page":"171","last_page":"181"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10300","display_name":"Congenital Heart Disease Studies","score":0.9878000020980835,"subfield":{"id":"https://openalex.org/subfields/2713","display_name":"Epidemiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10300","display_name":"Congenital Heart Disease Studies","score":0.9878000020980835,"subfield":{"id":"https://openalex.org/subfields/2713","display_name":"Epidemiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11055","display_name":"Cardiac and Coronary Surgery Techniques","score":0.002400000113993883,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11677","display_name":"Congenital heart defects research","score":0.0015999999595806003,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/kabuki-syndrome","display_name":"Kabuki syndrome","score":0.9879999756813049},{"id":"https://openalex.org/keywords/congenital-heart-surgery","display_name":"congenital heart surgery","score":0.9929999709129333},{"id":"https://openalex.org/keywords/postoperative-outcomes","display_name":"postoperative outcomes","score":0.8220000267028809},{"id":"https://openalex.org/keywords/norwood-procedure","display_name":"Norwood procedure","score":0.847000002861023},{"id":"https://openalex.org/keywords/postoperative-infection","display_name":"postoperative infection","score":0.878000020980835},{"id":"https://openalex.org/keywords/pleural-effusion","display_name":"pleural effusion","score":0.984000027179718},{"id":"https://openalex.org/keywords/survival","display_name":"survival","score":0.625},{"id":"https://openalex.org/keywords/genetic-syndromes","display_name":"genetic syndromes","score":0.8090000152587891},{"id":"https://openalex.org/keywords/pediatric-cardiac-surgery","display_name":"pediatric cardiac surgery","score":0.5519999861717224}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.977584958076477},{"id":"https://openalex.org/C2780074459","wikidata":"https://www.wikidata.org/wiki/Q389735","display_name":"Heart disease","level":2,"score":0.6187023520469666},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.5365450978279114},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.5297523736953735},{"id":"https://openalex.org/C2779545397","wikidata":"https://www.wikidata.org/wiki/Q1641399","display_name":"Hypoplastic left heart syndrome","level":3,"score":0.528239905834198},{"id":"https://openalex.org/C195910791","wikidata":"https://www.wikidata.org/wiki/Q1324077","display_name":"Medical record","level":2,"score":0.5275449752807617},{"id":"https://openalex.org/C2780084366","wikidata":"https://www.wikidata.org/wiki/Q37732","display_name":"Demographics","level":2,"score":0.4746026396751404},{"id":"https://openalex.org/C2909602630","wikidata":"https://www.wikidata.org/wiki/Q179630","display_name":"Genetic syndromes","level":2,"score":0.43537983298301697},{"id":"https://openalex.org/C2778789114","wikidata":"https://www.wikidata.org/wiki/Q235007","display_name":"Cardiac surgery","level":2,"score":0.4346843957901001},{"id":"https://openalex.org/C72563966","wikidata":"https://www.wikidata.org/wiki/Q1303415","display_name":"Cohort","level":2,"score":0.42468947172164917},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.19185411930084229},{"id":"https://openalex.org/C149923435","wikidata":"https://www.wikidata.org/wiki/Q37732","display_name":"Demography","level":1,"score":0.0},{"id":"https://openalex.org/C144024400","wikidata":"https://www.wikidata.org/wiki/Q21201","display_name":"Sociology","level":0,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.32604/chd.2021.014409","doi":"https://doi.org/10.32604/chd.2021.014409","is_oa":true,"landing_page_url":"https://doi.org/10.32604/chd.2021.014409","pdf_url":"https://www.techscience.com/ueditor/files/TSP_CHD-16-2/TSP_CHD_14409/TSP_CHD_14409.pdf","source":{"id":"https://openalex.org/S173391249","display_name":"Congenital Heart Disease","issn_l":"1747-079X","issn":["1747-079X","1747-0803"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","jufo-1","ki-jl-1","norway-1","oarl","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Congenital Heart Disease","raw_type":"journal-article"}],"best_oa_location":{"id":"doi:10.32604/chd.2021.014409","doi":"https://doi.org/10.32604/chd.2021.014409","is_oa":true,"landing_page_url":"https://doi.org/10.32604/chd.2021.014409","pdf_url":"https://www.techscience.com/ueditor/files/TSP_CHD-16-2/TSP_CHD_14409/TSP_CHD_14409.pdf","source":{"id":"https://openalex.org/S173391249","display_name":"Congenital Heart Disease","issn_l":"1747-079X","issn":["1747-079X","1747-0803"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","jufo-1","ki-jl-1","norway-1","oarl","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Congenital Heart Disease","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"sustainable_development_goals_aurora":[{"id":"https://metadata.un.org/sdg/3","score":0.8799999952316284,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W3123354792.pdf","grobid_xml":"https://content.openalex.org/works/W3123354792.grobid-xml"},"referenced_works_count":33,"referenced_works":["https://openalex.org/W1537101177","https://openalex.org/W1792586648","https://openalex.org/W1964948395","https://openalex.org/W1967289389","https://openalex.org/W1968837117","https://openalex.org/W1979300931","https://openalex.org/W1983571826","https://openalex.org/W1994073345","https://openalex.org/W1994441122","https://openalex.org/W1994715440","https://openalex.org/W1999966796","https://openalex.org/W2008353880","https://openalex.org/W2008769536","https://openalex.org/W2024319943","https://openalex.org/W2049671475","https://openalex.org/W2058330915","https://openalex.org/W2060329891","https://openalex.org/W2061311850","https://openalex.org/W2082162304","https://openalex.org/W2082487794","https://openalex.org/W2084224714","https://openalex.org/W2084620249","https://openalex.org/W2093554111","https://openalex.org/W2094501389","https://openalex.org/W2096788206","https://openalex.org/W2101215415","https://openalex.org/W2525919158","https://openalex.org/W2751637236","https://openalex.org/W2754547599","https://openalex.org/W2803181047","https://openalex.org/W2904996174","https://openalex.org/W4255436768","https://openalex.org/W4293241248"],"related_works":["https://openalex.org/W7213330590","https://openalex.org/W4407980803","https://openalex.org/W2120717348","https://openalex.org/W2069994610","https://openalex.org/W2097540964","https://openalex.org/W4321780082","https://openalex.org/W1514983847","https://openalex.org/W2972798301","https://openalex.org/W7098694688","https://openalex.org/W7096762293"],"abstract_inverted_index":{"Background:":[0],"Patients":[1,70],"with":[2,33,71,119,230],"genetic":[3,227],"syndromes":[4],"who":[5],"undergo":[6],"surgery":[7,30,66,141],"to":[8,48,67,85,209,241],"correct":[9],"congenital":[10,28,64,130,249],"heart":[11,29,65,250],"defects":[12],"can":[13],"be":[14,210,242],"at":[15,137,199,211,243],"risk":[16,213,245],"for":[17,157,163,188,214],"increased":[18,212,244],"morbidity":[19],"or":[20],"mortality.":[21],"Surgical":[22],"outcomes":[23,55],"and":[24,53,78,93,103,121,135,145,160,169,174,178,236],"postoperative":[25,51,219],"courses":[26,52],"following":[27,248],"in":[31,56,176],"patients":[32,62,117,207,224,229],"Kabuki-Syndrome":[34],"(KS)":[35],"have":[36],"not":[37,239],"been":[38],"well":[39],"studied.":[40],"Objectives:":[41],"The":[42],"purpose":[43],"of":[44,60,76,81,128,140,151,180,223,233,246],"this":[45],"study":[46],"was":[47,142,154,184,195,202],"describe":[49],"the":[50,57,109,138,164],"associated":[54],"largest":[58],"set":[59],"KS":[61,77,120,206,237],"undergoing":[63],"date.":[68],"Methods:":[69],"a":[72,79,82,122,215,226,231,234],"confirmed":[73],"molecular":[74],"diagnosis":[75,80,232],"CHD":[83,235],"admitted":[84],"Texas":[86],"Children\u2019s":[87],"Hospital":[88],"between":[89],"January":[90,94],"1,":[91,95],"2000":[92],"2020":[96],"were":[97,106,172],"included":[98],"(n":[99],"=":[100],"20).":[101],"Demographics":[102],"medical":[104],"histories":[105],"collected":[107],"from":[108],"hospitals\u2019":[110],"electronic":[111],"health":[112],"records.":[113],"Results:":[114],"Of":[115],"20":[116],"identified":[118],"CHD,":[123],"15":[124],"required":[125],"surgical":[126],"correction":[127],"their":[129],"cardiac":[131],"malformation.":[132],"Median":[133,149,191],"age":[134],"weight":[136],"time":[139,194],"2":[143],"months":[144],"4.1":[146],"kg,":[147],"respectively.":[148,182],"duration":[150],"hospital":[152],"stay":[153],"49":[155],"days":[156,162],"all":[158],"surgeries":[159],"151":[161],"Norwood":[165],"procedure.":[166],"Postoperative":[167],"infections":[168],"pleural":[170],"effusions":[171],"detected":[173],"treated":[175],"45.8%":[177],"50%":[179],"patients,":[181],"There":[183],"no":[185],"in-hospital":[186],"mortality":[187,247],"any":[189],"surgery.":[190,251],"follow":[192],"up":[193],"5.6":[196],"years;":[197],"survival":[198],"6":[200],"years":[201],"94%.":[203],"Conclusions:":[204],"Although":[205],"seem":[208],"more":[216],"complicated,":[217],"prolonged":[218],"course":[220],"than":[221],"that":[222],"without":[225],"syndrome,":[228],"do":[238],"appear":[240]},"counts_by_year":[{"year":2026,"cited_by_count":2},{"year":2025,"cited_by_count":1},{"year":2021,"cited_by_count":1}],"updated_date":"2026-10-30T12:39:25.321706","created_date":"2021-02-01T00:00:00"}