The aim of the study was to analyse critically the programme for surgical m
anagement of children in Papua New Guinea (PNG) with congenital heart disea
se. A hospital record-based analysis was undertaken to document the pattern
, management and short-term outcome of surgery in PNG children referred wit
h a diagnosis of congenital heart disease to the Royal Alexandra Hospital f
or Children in Sydney, Australia. On admission, physical examination, chest
radiogram, electrocardiogram, cross-sectional echocardiogram and, in most
cases, cardiac catheterization were performed. Of the 170 children referred
over the 17-year period, 1978-1994, 165 were confirmed to have congenital
heart disease and were included in the study. Their ages ranged from 2 mont
hs to 16 years (median 5.5) and the male to female ratio was 1:1. One-sixth
had delayed milestones and one-fifth long-term wasting. A large number wer
e tachypnoeic, in heart failure or had pulmonary hypertension on admission.
Ventricular septal defect, 34%, tetralogy of Fallot, 23%, and patent ductu
s arteriosus, 16.4%, were the predominant defects. Lesions such as aortic s
tenosis, coarctation of the aorta and transposition of the great arteries a
re under-represented. Altogether, 133 children (81%) had surgery; 75% were
open- and 25% closed-heart operations. The complications were unremarkable
and the mortality rate (6%) acceptable for the era. The programme was there
fore very successful for a small proportion of children born in PNG with co
ngenital heart disease.