title: Management Options and Outcomes for Neonatal Hypoplastic Left Heart Syndrome in the Early Twenty-First Century.
abstract: Without surgical treatment, neonatal hypoplastic left heart syndrome (HLHS) mortality in the first year of life exceeds 90 % and, in spite of improved surgical outcomes, many families still opt for non-surgical management. The purpose of this study was to investigate trends in neonatal HLHS management and to identify characteristics of patients who did not undergo surgical palliation. Neonates with HLHS were identified from a serial cross-sectional analysis using the Healthcare Cost and Utilization Project's Kids' Inpatient Database from 2000 to 2012. The primary analysis compared children undergoing surgical palliation to those discharged alive without surgery using a binary logistic regression model. Multivariate logistic regression was conducted to determine factors associated with treatment choice. A total of 1750 patients underwent analysis. Overall hospital mortality decreased from 35.3 % in 2000 to 22.9 % in 2012. The percentage of patients undergoing comfort care discharge without surgery also decreased from 21.2 to 14.8 %. After controlling for demographics and comorbidities, older patients at presentation were less likely to undergo surgery (OR 0.93, 0.91-0.96), and patients in 2012 were more likely to undergo surgery compared to those in prior years (OR 1.5, 1.1-2.1). Discharge without surgical intervention is decreasing with a 30 % reduction between 2000 and 2012. Given the improvement in surgical outcomes, further dialogue about ethical justification of non-operative comfort or palliative care is warranted. In the meantime, clinicians should present families with surgical outcome data and recommend intervention, while supporting their option to refuse.
authors.affiliation: Department of Pediatrics, Section of Critical Care Medicine, University of Chicago Medicine and Comer Children's Hospital, 5841 S. Maryland Avenue, MC 1145, Chicago, IL, 60637, USA. jasonk@uchicago.edu.
authors.forename: Jason M
authors.initials: JM
authors.lastname: Kane
authors.affiliation: Rush University Medical Center, Chicago, IL, USA.
authors.forename: Jeff
authors.initials: J
authors.lastname: Canar
authors.affiliation: Rush University Medical Center, Chicago, IL, USA.
authors.forename: Valerie
authors.initials: V
authors.lastname: Kalinowski
authors.affiliation: Rush University Medical Center, Chicago, IL, USA.
authors.forename: Tricia J
authors.initials: TJ
authors.lastname: Johnson
authors.affiliation: University of Kansas Medical Center, Kansas City, KS, USA.
authors.forename: K Sarah
authors.initials: KS
authors.lastname: Hoehn
mesh_terms: D015897:Comorbidity; D003430:Cross-Sectional Studies; D019468:Disease Management; D005260:Female; D017048:Health Care Costs / Q000639:trends; D016027:Heart Transplantation; D017052:Hospital Mortality / Q000639:trends*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000453:epidemiology* / Q000628:therapy*; D007231:Infant, Newborn; D007902:Length of Stay / Q000639:trends*; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D058327:Norwood Procedures / Q000009:adverse effects*; D010166:Palliative Care / Q000191:economics; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
keywords: Cardiology; Hypoplastic left heart syndrome; Norwood procedures; Palliative care; Pediatrics; Thoracic surgery