title: Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience.
abstract: OBJECTIVE
  Management of pulmonary atresia with intact ventricular septum (PAIVS) can be directed to either biventricular repair or univentricular palliation. The optimal management strategy has yet to be defined.
  
  
  METHODS
  All patients operated at the Royal Children's Hospital, Melbourne for PAIVS between 1990 and 2006 (n = 81) were reviewed. Patients were retrospectively stratified into a simple three-tiered classification based on right ventricle (RV) size. Multivariate logistic regression analysis was performed to identify risk factors of mortality.
  
  
  RESULTS
  The distribution of RV sizes was normal in 11 (14%), moderate hypoplasia in 45 (56%), and severe hypoplasia in 25 (31%) patients. RV-to-coronary-artery connections were present in 33 (41%) and RV coronary dependence in six patients (7%). Sixteen patients died (20%). The end-status of the remaining patients was biventricular repair in 31/81 (38%), 1½-ventricle repair in 10/81 (12%), Fontan circulation in 14/81 (17%), transplantation in 1/81 (1%), and still awaiting repair in 9/81 (11%). Ten-year survival was 80% (95% confidence interval (CI): 71-87%). Independent predictors of mortality were lower tricuspid valve (TV) annulus size Z-score and the presence of RV-to-coronary-artery connections.
  
  
  CONCLUSIONS
  A simple three-tiered classification based on RV size may allow initial stratification into biventricular or univentricular repair for patients with normal RV size and severe RV hypoplasia. In patients with moderate RV hypoplasia, the presence of RV-to-coronary-artery connections or a TV Z-score<-2 should caution one against attempting biventricular repair.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital and the Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Matthew
authors.initials: M
authors.lastname: Liava'a
authors.forename: Paul
authors.initials: P
authors.lastname: Brooks
authors.forename: Igor
authors.initials: I
authors.lastname: Konstantinov
authors.forename: Christian
authors.initials: C
authors.lastname: Brizard
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D001724:Birth Weight; D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000639:trends; D003326:Coronary Circulation / Q000502:physiology; D004812:Epidemiologic Methods; D005865:Gestational Age; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D010166:Palliative Care / Q000379:methods; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal; D054088:Ventricular Septum / Q000000981:diagnostic imaging / Q000473:pathology*