title: Predictors of Catastrophic Adverse Outcomes in Children With Pulmonary Hypertension Undergoing Cardiac Catheterization: A Multi-Institutional Analysis From the Pediatric Health Information Systems Database.
abstract: BACKGROUND
  Cardiac catheterization is the standard of care procedure for diagnosis, choice of therapy, and longitudinal follow-up of children and adults with pulmonary hypertension (PH). However, the procedure is invasive and has risks associated with both the procedure and recovery period.
  
  
  OBJECTIVE
  The purpose of this study was to identify risk factors for catastrophic adverse outcomes in children with PH undergoing cardiac catheterization.
  
  
  METHODS
  We studied children and young adults up to 21 years of age with PH undergoing 1 or more cardiac catheterization at centers participating in the Pediatric Health Information Systems database between 2007 and 2012. Using mixed-effects multivariable regression, we assessed the association between pre-specified subject- and procedure-level covariates and the risk of the composite outcome of death or initiation of mechanical circulatory support within 1 day of cardiac catheterization after adjustment for patient- and procedure-level factors.
  
  
  RESULTS
  A total of 6,339 procedures performed on 4,401 patients with a diagnosis of PH from 38 of 43 centers contributing data to the Pediatric Health Information Systems database were included. The observed risk of composite outcome was 3.5%. In multivariate modeling, the adjusted risk of the composite outcome was 3.3%. Younger age at catheterization, cardiac operation in the same admission as the catheterization, pre-procedural systemic vasodilator infusion, and hemodialysis were independently associated with an increased risk of adverse outcomes. Pre-procedural use of pulmonary vasodilators was associated with reduced risk of composite outcome.
  
  
  CONCLUSIONS
  The risk of cardiac catheterization in children and young adults with PH is high relative to previously reported risk in other pediatric populations. The risk is influenced by patient-level factors. Further research is necessary to determine whether knowledge of these factors can be translated into practices that improve outcomes for children with PH.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address: obyrne@post.harvard.edu.
authors.forename: Michael L
authors.initials: ML
authors.lastname: O'Byrne
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Glatz
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Brian D
authors.initials: BD
authors.lastname: Hanna
authors.affiliation: Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Russell T
authors.initials: RT
authors.lastname: Shinohara
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Gillespie
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Yoav
authors.initials: Y
authors.lastname: Dori
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Jonathan J
authors.initials: JJ
authors.lastname: Rome
authors.affiliation: Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Penn Cardiovascular Institute, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
mesh_terms: D000293:Adolescent; D006328:Cardiac Catheterization / Q000009:adverse effects* / Q000401:mortality*; D002388:Catastrophic Illness / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual / Q000639:trends; D005260:Female; D063005:Health Information Systems / Q000639:trends; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Pediatric Health Information Systems Database; extracorporeal membrane oxygenation; intervention; mortality; outcomes research; pediatric cardiology