title: Pulmonary atresia with intact ventricular septum: limitations of catheter-based intervention.
abstract: BACKGROUND
  Pulmonary atresia with intact ventricular septum (PAIVS) has a wide spectrum of anatomic heterogeneity and invokes a wide variety of treatment strategies. We reviewed the outcome of our patients with PAIVS in order to delineate strategies for the optimal management of PAIVS. In particular, the possibility of avoiding neonatal surgical intervention with catheter-based technology was assessed.
  
  
  METHODS
  The study cohort was composed of all patients presented with PAIVS from January 1999 through December 2005. Demographic and anatomic variables were analyzed to determine association with in-hospital mortality.
  
  
  RESULTS
  Forty-four infants with PAIVS underwent catheter valvuloplasty (n = 17) and (or) surgical intervention (n = 42). The mean age and weight of the infants was six days and 3.1 kg, and the average follow-up was 40 +/- 29.5 months. Five (11%) had right ventricle dependent coronary circulation (RVDCC) and six (14%) had Ebstein's anomaly. Five (11%) patients died. Of those who underwent catheter valvotomy, three (18%) underwent shunt placement, 12 (71%) underwent right ventricular outflow tract reconstruction with shunt placement, and only two (12%) did not require a further surgical intervention in the newborn period. Multivariable analyses demonstrated RVDCC (odds ratio 21.3, p = 0.025) and Ebstein's anomaly (odds ratio 16.0, p = 0.038) to be risk factors for in-hospital mortality. Of those patients with Ebstein's anomaly, a single ventricle approach had a better outcome.
  
  
  CONCLUSIONS
  We demonstrated excellent recent outcomes for patients with PAIVS. Catheter-based interventions rarely avoid surgical repair. The RVDCC and Ebstein's anomaly were associated with high mortality. In patients with Ebstein's anomaly, single ventricular pathway may be the better strategy for this specific patient population.
authors.affiliation: The Division of Pediatric Cardiac Surgery, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA. yh2240@columbia.edu
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Hirata
authors.forename: Jonathan M
authors.initials: JM
authors.lastname: Chen
authors.forename: Jan M
authors.initials: JM
authors.lastname: Quaegebeur
authors.forename: William E
authors.initials: WE
authors.lastname: Hellenbrand
authors.forename: Ralph S
authors.initials: RS
authors.lastname: Mosca
mesh_terms: D001164:Arteriovenous Fistula / Q000453:epidemiology; D001724:Birth Weight; D006328:Cardiac Catheterization / Q000295:instrumentation / Q000379:methods*; D015331:Cohort Studies; D003330:Coronary Vessel Anomalies / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000453:epidemiology; D006346:Heart Septum / Q000033:anatomy & histology*; D006352:Heart Ventricles / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007258:Informed Consent; D018633:Pulmonary Atresia / Q000145:classification / Q000401:mortality / Q000601:surgery* / Q000628:therapy*; D015203:Reproducibility of Results; D012189:Retrospective Studies; D016019:Survival Analysis; D014261:Tricuspid Valve / Q000033:anatomy & histology