Morphology of pulmonary atresia with ventricular septum in patients dying after operation

Abstract

In 1976, we adopted staged surgical management of pulmonary atresia with intact ventricular septum: stage 1 = establishment of a systemic to pulmonary artery shunt; stage 2 = open reconstruction of the right ventricular outflow tract and pulmonary valve; and stage 3 = closure of the shunt and interatrial communication. The morphological features of nine specimens obtained from 10 patients who died were reviewed. Special attention was given to features that might have influenced the poor surgical outcome in these patients. Survival after stage 1 depends on adequate systemic to pulmonary artery blood flow, initially as a combination of ductus arteriosus and shunt flow, with subsequent modification if the ductus closes. After stage 2, survival is influenced by left ventricular function and mitral valve function. The success of final correction (stage 3) depends largely on the morphology of both ventricles and their atrioventricular valves. It appears that the behavior of the ductus arteriosus and the size of the shunt are of vital importance for the survival of the infant. In 3 of the specimens, no right ventricular outflow tract was present, and in 2 others, short chordal attachments of the mitral valve were observed. Staged surgical correction appears to be a satisfactory approach if these considerations are taken into account.