title: Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum.
abstract: OBJECTIVE
  We sought to define the prevalence of definitive end states and their determinants in children given a diagnosis of pulmonary atresia and intact ventricular septum during the neonatal period.
  
  
  METHODS
  Between 1987 and 1997, 408 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective study by 33 institutions. Competing risks analysis was used to demonstrate the prevalence of 6 end states. Factors predictive of attaining each end state were identified by means of multivariable analysis with bootstrap validation.
  
  
  RESULTS
  Overall survival was 77% at 1 month, 70% at 6 months, 60% at 5 years, and 58% at 15 years. Prevalence of end states 15 years after entry were as follows: 2-ventricle repair, 33%; Fontan repair, 20%; 1.5-ventricle repair, 5%; heart transplant, 2%; death before reaching definitive repair, 38%; and alive without definitive repair, 2%. Patient-related factors discriminating among end states primarily included adequacy of right-sided heart structures, degree of aberration of coronary circulation, low birth weight, and tricuspid valve regurgitation. After adjusting for these factors, 2 institutions were predictive of 2-ventricle repair, 1 of Fontan repair, and 6 of death before definitive repair. Two institutions were predictive of both 2-ventricle and Fontan repair. These 2 institutions achieved a higher risk-adjusted prevalence of definitive repair and a lower prevalence of prerepair mortality.
  
  
  CONCLUSIONS
  Characteristics of neonates with pulmonary atresia and intact ventricular septum predict type of definitive repair. A morphologically driven institutional protocol emphasizing both 2-ventricle and Fontan pathways might mitigate the negative effect of unfavorable morphology. In the current era, 85% of neonates are likely to reach a definitive surgical end point, with 2-ventricle repair achieved in an estimated 50%.
authors.affiliation: Division of Cardiothoracic Surgery, Hospital for Sick Children, Toronto, Onario, Canada.
authors.forename: David A
authors.initials: DA
authors.lastname: Ashburn
authors.forename: Eugene H
authors.initials: EH
authors.lastname: Blackstone
authors.forename: Winfield J
authors.initials: WJ
authors.lastname: Wells
authors.forename: Richard A
authors.initials: RA
authors.lastname: Jonas
authors.forename: Frank A
authors.initials: FA
authors.lastname: Pigula
authors.forename: Peter B
authors.initials: PB
authors.lastname: Manning
authors.forename: Gary K
authors.initials: GK
authors.lastname: Lofland
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
mesh_terms: D006348:Cardiac Surgical Procedures / Q000145:classification*; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006346:Heart Septum / Q000601:surgery; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007229:Infant Welfare; D007231:Infant, Newborn; D015999:Multivariate Analysis; D009656:North America / Q000453:epidemiology; D011237:Predictive Value of Tests; D015995:Prevalence; D011446:Prospective Studies; D018633:Pulmonary Atresia / Q000401:mortality* / Q000601:surgery*; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome