title: Surgical programme at Royal Alexandra Hospital, Sydney, for Papua New Guinea children with congenital heart disease, 1978-1994.
abstract: OBJECTIVE
  To report the history of the Royal Alexandra Hospital for Children (RAHC) Papua New Guinea (PNG) cardiac surgical programme and describe the selection, preoperative clinical features and postoperative outcome of children with congenital heart disease managed by the programme.
  
  
  METHODS
  Details for each of the PNG cardiac patients admitted to RAHC following selection by visiting cardiologists between 1978 and 1994 were entered into a database, and analysed and interpreted.
  
  
  RESULTS
  A congenital heart defect was confirmed in 165 of the 170 children selected. The male to female ratio was 1:1 and the mean age on admission to RAHC was 5.5 years. Almost all of the children for whom data were available (98%) had a weight for age and 41% had a height for age less than the 3rd centile. One-sixth had delayed milestones. A large number were tachypnoeic, in heart failure, or had pulmonary hypertension on admission. Ventricular septal defect and tetralogy of Fallot were the commonest defects, and lesions such as aortic stenosis, coarctation of the aorta and transposition of the great arteries were absent or rare. Thirty-one (19%) of the children selected initially did not receive surgery because of pulmonary hypertension, or because the lesions did not fall within the programme guidelines for operation. One hundred and twenty-nine children had corrective and four had palliative procedures. Half of the operated children had postoperative complications. Eight children died, all following open-heart procedures, giving a case fatality rate of 6%. Preoperative tachypnoea, hepatomegaly, cardiac failure and pulmonary hypertension were strongly associated with poor outcome.
  
  
  CONCLUSIONS
  The programme was an arduous exercise for all organizations concerned, but achieved comparatively good short-term outcomes. The experience gained should assist in planning for similar programmes.
authors.affiliation: Clinical Sciences Division, School of Medicine, University of Papua New Guinea, Boroko, Australia.
authors.forename: N
authors.initials: N
authors.lastname: Tefuarani
authors.forename: R
authors.initials: R
authors.lastname: Hawker
authors.forename: J
authors.initials: J
authors.lastname: Vince
authors.forename: A
authors.initials: A
authors.lastname: Sleigh
authors.forename: G M
authors.initials: GM
authors.lastname: Williams
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006786:Hospitals, Urban; D006801:Humans; D007223:Infant; D007902:Length of Stay; D008137:Longitudinal Studies; D008297:Male; D009517:New South Wales; D010043:Outcome and Process Assessment, Health Care; D010219:Papua New Guinea / Q000208:ethnology; D011183:Postoperative Complications; D015397:Program Evaluation; D013503:Surgery Department, Hospital / Q000458:organization & administration*