The plain chest radiographs of 40 patients with the absent pulmonary v
alve syndrome in association with tetralogy of Fallot have been review
ed. The conventional frontal projection showed characteristic changes
of massively dilated central pulmonary arteries with normal peripheral
markings in all patients (100%). Lobar hyperinflation was present in
a significant proportion of the infant group (38%) which in a smaller
group can be a complication requiring surgery in addition to that for
the cardiac malformation.