{"id":"https://openalex.org/W1895944121","doi":"https://doi.org/10.1111/pan.12775","title":"Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review","display_name":"Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review","publication_year":2015,"publication_date":"2015-10-12","ids":{"openalex":"https://openalex.org/W1895944121","doi":"https://doi.org/10.1111/pan.12775","mag":"1895944121","pmid":"https://pubmed.ncbi.nlm.nih.gov/26456018"},"language":"en","primary_location":{"id":"doi:10.1111/pan.12775","doi":"https://doi.org/10.1111/pan.12775","is_oa":false,"landing_page_url":"https://doi.org/10.1111/pan.12775","pdf_url":null,"source":{"id":"https://openalex.org/S192565471","display_name":"Pediatric Anesthesia","issn_l":"1155-5645","issn":["1155-5645","1460-9592"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","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":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatric Anesthesia","raw_type":"journal-article"},"volume":null,"type":"review","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5082565342","display_name":"Andrew Matisoff","orcid":"https://orcid.org/0000-0002-1169-0719","observed_orcids":["https://orcid.org/0000-0002-1169-0719"]},"institutions":[{"id":"https://openalex.org/I1336742384","display_name":"Children's National","ror":"https://ror.org/03wa2q724","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1336742384"]},{"id":"https://openalex.org/I193531525","display_name":"George Washington University","ror":"https://ror.org/00y4zzh67","country_code":"US","type":"education","lineage":["https://openalex.org/I193531525"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Andrew J. Matisoff","raw_affiliation_strings":["Division of Anesthesia, Sedation and Perioperative Medicine, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Anesthesia, Sedation and Perioperative Medicine, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA","institution_ids":["https://openalex.org/I1336742384","https://openalex.org/I193531525"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5075009634","display_name":"Laura Olivieri","orcid":"https://orcid.org/0000-0002-3783-0783","observed_orcids":["https://orcid.org/0000-0002-3783-0783"]},"institutions":[{"id":"https://openalex.org/I1336742384","display_name":"Children's National","ror":"https://ror.org/03wa2q724","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1336742384"]},{"id":"https://openalex.org/I193531525","display_name":"George Washington University","ror":"https://ror.org/00y4zzh67","country_code":"US","type":"education","lineage":["https://openalex.org/I193531525"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Laura Olivieri","raw_affiliation_strings":["Division of Cardiology, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Cardiology, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA","institution_ids":["https://openalex.org/I1336742384","https://openalex.org/I193531525"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5021168505","display_name":"Jamie McElrath Schwartz","orcid":"https://orcid.org/0000-0003-3208-4897","observed_orcids":["https://orcid.org/0000-0003-3208-4897"]},"institutions":[{"id":"https://openalex.org/I1336742384","display_name":"Children's National","ror":"https://ror.org/03wa2q724","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1336742384"]},{"id":"https://openalex.org/I193531525","display_name":"George Washington University","ror":"https://ror.org/00y4zzh67","country_code":"US","type":"education","lineage":["https://openalex.org/I193531525"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Jamie M. Schwartz","raw_affiliation_strings":["Division of Anesthesia, Sedation and Perioperative Medicine, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA","Division of Critical Care, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Anesthesia, Sedation and Perioperative Medicine, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA","institution_ids":["https://openalex.org/I1336742384","https://openalex.org/I193531525"]},{"raw_affiliation_string":"Division of Critical Care, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA","institution_ids":["https://openalex.org/I1336742384","https://openalex.org/I193531525"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5013500759","display_name":"Nina Deutsch","orcid":"https://orcid.org/0000-0001-7137-4737","observed_orcids":["https://orcid.org/0000-0001-7137-4737"]},"institutions":[{"id":"https://openalex.org/I1336742384","display_name":"Children's National","ror":"https://ror.org/03wa2q724","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1336742384"]},{"id":"https://openalex.org/I193531525","display_name":"George Washington University","ror":"https://ror.org/00y4zzh67","country_code":"US","type":"education","lineage":["https://openalex.org/I193531525"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Nina Deutsch","raw_affiliation_strings":["Division of Anesthesia, Sedation and Perioperative Medicine, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Anesthesia, Sedation and Perioperative Medicine, Children's National Health System, George Washington University School of Medicine, Washington, DC, USA","institution_ids":["https://openalex.org/I1336742384","https://openalex.org/I193531525"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5082565342"],"corresponding_institution_ids":["https://openalex.org/I1336742384","https://openalex.org/I193531525"],"apc_list":{"value":4080,"currency":"USD","value_usd":4080},"apc_paid":null,"fwci":0.7514,"has_fulltext":false,"cited_by_count":79,"citation_normalized_percentile":{"value":0.53815261,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":94,"max":99},"biblio":{"volume":"25","issue":"12","first_page":"1207","last_page":"1215"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T13258","display_name":"Williams Syndrome Research","score":0.9940999746322632,"subfield":{"id":"https://openalex.org/subfields/2806","display_name":"Developmental Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T13258","display_name":"Williams Syndrome Research","score":0.9940999746322632,"subfield":{"id":"https://openalex.org/subfields/2806","display_name":"Developmental Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10436","display_name":"Anesthesia and Sedative Agents","score":0.0017999999690800905,"subfield":{"id":"https://openalex.org/subfields/2703","display_name":"Anesthesiology and Pain Medicine"},"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/T10300","display_name":"Congenital Heart Disease Studies","score":0.0013000000035390258,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/williams-syndrome","display_name":"Williams syndrome","score":0.9829999804496765},{"id":"https://openalex.org/keywords/anesthetic-risk-assessment","display_name":"anesthetic risk assessment","score":0.8109999895095825},{"id":"https://openalex.org/keywords/supravalvular-aortic-stenosis","display_name":"supravalvular aortic stenosis","score":0.9509999752044678},{"id":"https://openalex.org/keywords/coronary-artery-anomalies","display_name":"coronary artery anomalies","score":0.9789999723434448},{"id":"https://openalex.org/keywords/myocardial-ischemia","display_name":"myocardial ischemia","score":0.8610000014305115},{"id":"https://openalex.org/keywords/periprocedural-management","display_name":"periprocedural management","score":0.8320000171661377}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9456431865692139},{"id":"https://openalex.org/C2778162923","wikidata":"https://www.wikidata.org/wiki/Q4990531","display_name":"Anesthetic","level":2,"score":0.6777921319007874},{"id":"https://openalex.org/C2776814716","wikidata":"https://www.wikidata.org/wiki/Q574801","display_name":"Sedation","level":2,"score":0.5665429830551147},{"id":"https://openalex.org/C2908647359","wikidata":"https://www.wikidata.org/wiki/Q2625603","display_name":"Population","level":2,"score":0.5004665851593018},{"id":"https://openalex.org/C2781240822","wikidata":"https://www.wikidata.org/wiki/Q558077","display_name":"Williams syndrome","level":3,"score":0.43792468309402466},{"id":"https://openalex.org/C42219234","wikidata":"https://www.wikidata.org/wiki/Q131130","display_name":"Anesthesia","level":1,"score":0.40958744287490845},{"id":"https://openalex.org/C177713679","wikidata":"https://www.wikidata.org/wiki/Q679690","display_name":"Intensive care medicine","level":1,"score":0.40412548184394836},{"id":"https://openalex.org/C164705383","wikidata":"https://www.wikidata.org/wiki/Q10379","display_name":"Cardiology","level":1,"score":0.38362666964530945},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3541296124458313},{"id":"https://openalex.org/C118552586","wikidata":"https://www.wikidata.org/wiki/Q7867","display_name":"Psychiatry","level":1,"score":0.12486532330513},{"id":"https://openalex.org/C169900460","wikidata":"https://www.wikidata.org/wiki/Q2200417","display_name":"Cognition","level":2,"score":0.0},{"id":"https://openalex.org/C99454951","wikidata":"https://www.wikidata.org/wiki/Q932068","display_name":"Environmental health","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D000758","descriptor_name":"Anesthesia","qualifier_ui":"Q000009","qualifier_name":"adverse effects","is_major_topic":false},{"descriptor_ui":"D000758","descriptor_name":"Anesthesia","qualifier_ui":"Q000379","qualifier_name":"methods","is_major_topic":true},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006323","descriptor_name":"Heart Arrest","qualifier_ui":"Q000139","qualifier_name":"chemically induced","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007431","descriptor_name":"Intraoperative Complications","qualifier_ui":"Q000517","qualifier_name":"prevention & control","is_major_topic":false},{"descriptor_ui":"D018570","descriptor_name":"Risk Assessment","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D018980","descriptor_name":"Williams Syndrome","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D018980","descriptor_name":"Williams Syndrome","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":false},{"descriptor_ui":"D019990","descriptor_name":"Perioperative Care","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1111/pan.12775","doi":"https://doi.org/10.1111/pan.12775","is_oa":false,"landing_page_url":"https://doi.org/10.1111/pan.12775","pdf_url":null,"source":{"id":"https://openalex.org/S192565471","display_name":"Pediatric Anesthesia","issn_l":"1155-5645","issn":["1155-5645","1460-9592"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","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":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatric Anesthesia","raw_type":"journal-article"},{"id":"pmid:26456018","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/26456018","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Paediatric anaesthesia","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":41,"referenced_works":["https://openalex.org/W30994966","https://openalex.org/W1601069617","https://openalex.org/W1934158103","https://openalex.org/W1965187539","https://openalex.org/W1969800606","https://openalex.org/W1976563428","https://openalex.org/W1983955929","https://openalex.org/W1987085341","https://openalex.org/W1993943660","https://openalex.org/W1996406138","https://openalex.org/W2008166400","https://openalex.org/W2008818895","https://openalex.org/W2034314926","https://openalex.org/W2037554811","https://openalex.org/W2043555103","https://openalex.org/W2049530585","https://openalex.org/W2051684775","https://openalex.org/W2058412576","https://openalex.org/W2061725634","https://openalex.org/W2065066667","https://openalex.org/W2075664988","https://openalex.org/W2076046949","https://openalex.org/W2079922738","https://openalex.org/W2085897113","https://openalex.org/W2093998432","https://openalex.org/W2094733838","https://openalex.org/W2096500639","https://openalex.org/W2117768135","https://openalex.org/W2120151950","https://openalex.org/W2129992176","https://openalex.org/W2131300215","https://openalex.org/W2139592264","https://openalex.org/W2141152924","https://openalex.org/W2160274987","https://openalex.org/W2312737623","https://openalex.org/W2337043813","https://openalex.org/W2397919501","https://openalex.org/W2419571649","https://openalex.org/W2486730898","https://openalex.org/W2985215241","https://openalex.org/W4212878078"],"related_works":["https://openalex.org/W3001291580","https://openalex.org/W2080196787","https://openalex.org/W2461702284","https://openalex.org/W2793468906","https://openalex.org/W4412710549","https://openalex.org/W2085897113","https://openalex.org/W2605295287","https://openalex.org/W2298813389","https://openalex.org/W2298259541","https://openalex.org/W3201359835"],"abstract_inverted_index":{"Since":[0],"the":[1,54,120,136],"first":[2],"description":[3],"in":[4,18,38,110],"1961,":[5],"several":[6,80],"case":[7],"reports":[8],"have":[9,67,103],"documented":[10],"an":[11,33],"increased":[12,68],"incidence":[13],"of":[14,56,70,90,123,145],"anesthesia-related":[15],"cardiac":[16,40,92],"arrest":[17,93],"patients":[19,61,112],"with":[20,62,113],"Williams-Beuren":[21],"syndrome,":[22],"commonly":[23],"known":[24,65],"as":[25],"Williams":[26],"syndrome":[27],"(WS).":[28],"Widespread":[29],"arteriopathy":[30],"secondary":[31],"to":[32,66,95,130],"elastin":[34],"gene":[35],"defect":[36],"results":[37],"various":[39],"defects,":[41],"including":[42],"supravalvar":[43],"aortic":[44],"stenosis":[45],"(SVAS)":[46],"and":[47,75,88,139],"coronary":[48],"artery":[49],"anomalies,":[50],"which":[51],"can":[52],"increase":[53],"risk":[55,69,109,133],"myocardial":[57],"ischemia.":[58],"Even":[59],"though":[60],"WS":[63],"are":[64],"adverse":[71],"events":[72],"during":[73,85],"anesthesia":[74,84],"sedation,":[76],"they":[77],"often":[78],"undergo":[79],"procedures":[81],"that":[82,106],"require":[83],"their":[86],"lifetimes,":[87],"cases":[89],"perianesthetic":[91],"continue":[94],"be":[96],"reported.":[97],"To":[98],"date,":[99],"no":[100],"prospective":[101],"studies":[102],"been":[104],"reported":[105],"quantify":[107],"anesthetic":[108,132],"individual":[111],"WS.":[114],"In":[115],"this":[116,146],"article,":[117],"we":[118],"review":[119],"clinical":[121],"manifestations":[122],"WS,":[124],"propose":[125],"a":[126],"consensus,":[127],"expert-informed":[128],"method":[129],"estimate":[131],"based":[134],"on":[135],"current":[137],"literature,":[138],"provide":[140],"recommendations":[141],"for":[142],"periprocedural":[143],"management":[144],"patient":[147],"population.":[148]},"counts_by_year":[{"year":2026,"cited_by_count":5},{"year":2025,"cited_by_count":2},{"year":2024,"cited_by_count":6},{"year":2023,"cited_by_count":9},{"year":2022,"cited_by_count":9},{"year":2021,"cited_by_count":9},{"year":2020,"cited_by_count":8},{"year":2019,"cited_by_count":12},{"year":2018,"cited_by_count":9},{"year":2017,"cited_by_count":5},{"year":2016,"cited_by_count":4}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2016-06-24T00:00:00"}