Plain chest radiographic changes of the absent pulmonary valve syndrome

The plain chest radiographs of 40 patients with the absent pulmonary valve syndrome in association with tetralogy of Fallot have been reviewed. 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.