Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum

J Thorac Cardiovasc Surg. 2004 Apr;127(4):1000-7; discussion 1007-8. doi: 10.1016/j.jtcvs.2003.11.057.


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%.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cardiac Surgical Procedures / classification*
  • Follow-Up Studies
  • Fontan Procedure
  • Heart Septum / surgery
  • Heart Ventricles / surgery
  • Humans
  • Infant Welfare
  • Infant, Newborn
  • Multivariate Analysis
  • North America / epidemiology
  • Predictive Value of Tests
  • Prevalence
  • Prospective Studies
  • Pulmonary Atresia / mortality*
  • Pulmonary Atresia / surgery*
  • Risk Factors
  • Time Factors
  • Treatment Outcome