title: Haemodynamic characterisation and heart catheterisation complications in children with pulmonary hypertension: Insights from the Global TOPP Registry (tracking outcomes and practice in paediatric pulmonary hypertension).
abstract: BACKGROUND
  The TOPP Registry has been designed to provide epidemiologic, diagnostic, clinical, and outcome data on children with pulmonary hypertension (PH) confirmed by heart catheterisation (HC). This study aims to identify important characteristics of the haemodynamic profile at diagnosis and HC complications of paediatric patients presenting with PH.
  
  
  RESULTS
  HC data sets underwent a blinded review for confirmation of PH (defined as mean pulmonary arterial pressure ≥ 25 mmHg, pulmonary capillary wedge pressure ≤ 12 mmHg and pulmonary vascular resistance index [PVRI] of >3 WU × m(2)). Of 568 patients enrolled, 472 who fulfilled the inclusion criteria and had sufficient data from HC were analysed. A total of 908 diagnostic and follow-up HCs were performed and complications occurred in 5.9% of all HCs including five (0.6%) deaths. General anaesthesia (GA) was used in 53%, and conscious sedation in 47%. Complications at diagnosis were more likely to occur if GA was used (p=0.04) and with higher functional class (p=0.02). Mean cardiac index (CI) was within normal limits at diagnosis when analysed for the entire group (3.7 L/min/m(2); 95% confidence interval 3.4-4.1), as was right atrial pressure despite a severely increased PVRI (16.6 WU × m(2,) 95% confidence interval 15.6-17.76). However, 24% of the patients had a CI of <2.5L/min/m(2) at diagnosis. A progressive increase in PVRI and decrease in CI was observed with age (p<0.001).
  
  
  CONCLUSIONS
  In TOPP, haemodynamic assessment was remarkable for preserved CI in the majority of patients despite severely elevated PVRI. HC-related complication incidence was 5.9%, and was associated with GA and higher functional class.
authors.affiliation: Pediatric Cardiology, Department of the Child and Adolescents, Hôpital des Enfants, University of Geneva, Switzerland. Electronic address: maurice.beghetti@hcuge.ch.
authors.forename: M
authors.initials: M
authors.lastname: Beghetti
authors.affiliation: Cardiac Unit, Great Ormond Street Hospital for Children, London, UK.
authors.forename: I
authors.initials: I
authors.lastname: Schulze-Neick
authors.affiliation: Center for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Netherlands.
authors.forename: R M F
authors.initials: RM
authors.lastname: Berger
authors.affiliation: Pediatrics, University of Colorado School of Medicine, Aurora, USA.
authors.forename: D D
authors.initials: DD
authors.lastname: Ivy
authors.affiliation: M3C-Paediatric Cardiology, Université Paris Descartes, Necker Enfants Malades, AP-HP, Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Bonnet
authors.affiliation: Royal Children's Hospital, Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: R G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Toho University Medical Center Omori Hospital, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Saji
authors.affiliation: Seattle Children's Hospital, University of Washington School of Medicine, Seattle, USA.
authors.forename: D
authors.initials: D
authors.lastname: Yung
authors.affiliation: Texas Children's Hospital, Baylor College of Medicine, Houston, USA.
authors.forename: G B
authors.initials: GB
authors.lastname: Mallory
authors.affiliation: Innsbruck Medical University, Pediatric Cardiology, Innsbruck, Austria.
authors.forename: R
authors.initials: R
authors.lastname: Geiger
authors.affiliation: Children's National Medical Center, Pediatric Critical Care and Cardiology, WA, USA.
authors.forename: J T
authors.initials: JT
authors.lastname: Berger
authors.affiliation: Pediatrics, Columbia University, New York, USA.
authors.forename: R J
authors.initials: RJ
authors.lastname: Barst
authors.affiliation: Cardiology and Critical Care, University of Toronto, Toronto, ON, Canada.
authors.forename: T
authors.initials: T
authors.lastname: Humpl
mesh_terms: D000293:Adolescent; D006328:Cardiac Catheterization / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D014943:Global Health; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000503:physiopathology*; D007223:Infant; D008297:Male; D017063:Outcome Assessment, Health Care; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000293:injuries / Q000503:physiopathology*; D012042:Registries; D018570:Risk Assessment / Q000379:methods*; D013997:Time Factors
keywords: Catheterisation; Congenital; Heart defects; Hypertension; Paediatrics; Pulmonary