title: Variation in preoperative and intraoperative care for first-stage palliation of single-ventricle heart disease: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.
abstract: METHODS
  As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry.
  
  
  RESULTS
  From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9-5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia.
  
  
  CONCLUSIONS
  In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.
authors.affiliation: Department of Cardiology, Children's Hospital Boston, 300 Longwood Ave, Boston, MA 02115, USA. david.brown@cardio.chboston.org
authors.forename: David W
authors.initials: DW
authors.lastname: Brown
authors.forename: Jean A
authors.initials: JA
authors.lastname: Connor
authors.forename: Frank A
authors.initials: FA
authors.lastname: Pigula
authors.forename: Kudret
authors.initials: K
authors.lastname: Usmani
authors.forename: Thomas S
authors.initials: TS
authors.lastname: Klitzner
authors.forename: Robert H
authors.initials: RH
authors.lastname: Beekman
authors.forename: John D
authors.initials: JD
authors.lastname: Kugler
authors.forename: Gerard R
authors.initials: GR
authors.lastname: Martin
authors.forename: Steven R
authors.initials: SR
authors.lastname: Neish
authors.forename: Geoffrey L
authors.initials: GL
authors.lastname: Rosenthal
authors.forename: Carole
authors.initials: C
authors.lastname: Lannon
authors.forename: Kathy J
authors.initials: KJ
authors.lastname: Jenkins
mesh_terms: D026683:Advisory Committees / Q000458:organization & administration / Q000592:standards*; D000553:Ambulatory Care / Q000458:organization & administration / Q000592:standards; D002309:Cardiology / Q000458:organization & administration / Q000592:standards*; D003299:Cooperative Behavior; D019033:Delivery of Health Care, Integrated / Q000458:organization & administration / Q000592:standards*; D019317:Evidence-Based Medicine / Q000592:standards; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D033183:Interdisciplinary Communication; D007430:Intraoperative Care / Q000592:standards; D008297:Male; D058327:Norwood Procedures / Q000592:standards*; D009937:Organizational Objectives; D010166:Palliative Care / Q000592:standards; D018579:Patient Selection; D017410:Practice Guidelines as Topic / Q000592:standards; D011300:Preoperative Care / Q000592:standards; D058996:Quality Improvement / Q000458:organization & administration / Q000592:standards*; D012042:Registries; D012955:Societies, Medical / Q000458:organization & administration / Q000592:standards*; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States