title: Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients.
abstract: OBJECTIVE
  We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998.
  
  
  BACKGROUND
  Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair.
  
  
  METHODS
  We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports.
  
  
  RESULTS
  The mean initial Z-score for the diameter of the tricuspid valve was -0.99 +/- 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up.
  
  
  CONCLUSIONS
  The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.
authors.affiliation: Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Canada. dyamenahalliumesh@uams.edu
authors.forename: Umesh
authors.initials: U
authors.lastname: Dyamenahalli
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.forename: Cathy
authors.initials: C
authors.lastname: McDonald
authors.forename: Kalyani R
authors.initials: KR
authors.lastname: Trivedi
authors.forename: Jeffrey F
authors.initials: JF
authors.lastname: Smallhorn
authors.forename: Lee N
authors.initials: LN
authors.lastname: Benson
authors.forename: John
authors.initials: J
authors.lastname: Coles
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.forename: Robert M
authors.initials: RM
authors.lastname: Freedom
mesh_terms: D006348:Cardiac Surgical Procedures; D015331:Cohort Studies; D004437:Ebstein Anomaly / Q000601:surgery; D004452:Echocardiography; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000401:mortality / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D018633:Pulmonary Atresia / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome