Juxtaductal pulmonary artery coarctation. An underestimated cause of branch pulmonary artery stenosis in patients with pulmonary atresia or stenosis and a ventricular septal defect.

An angiographic and clinical study was performed to establish the prevalence of juxtaductal pulmonary artery coarctations in patients with pulmonary atresia or stenosis and a ventricular septal defect or a complex intracardiac defect. The present study is an adjunct to a previously reported portmortem study, in which the incidence of these pulmonary artery coarctations was found to be unexpectedly high. Pulmonary artery coarctations were identified angiographically in 10 of the 15 patients with pulmonary atresia. One additional patient had a bilateral ductus arteriosus and confluent pulmonary arteries, but did not have a pulmonary artery coarctation. Pulmonary artery coarctations were much less prevalent in the cases with pulmonary stenosis (5/50). However, these pulmonary artery coarctations appeared identical to those of the cases with pulmonary atresia. Fourteen pulmonary artery coarctations were located in the pulmonary artery at the side of the ductus arteriosus; this was left sided in 12 and right sided in two patients. In one patient the side of the ductus could not be established. The types and the locations of the pulmonary artery coarctations in the present study were identical to those in the previous postmortem study. Ductal tissue was found in many of the pulmonary artery coarctations of the postmortem study and is likely to be present in the clinical cases as well. The majority of the angiographically identified pulmonary artery coarctations were subsequently confirmed at operation or at autopsy. The clinical outcome and follow-up of the patients is discussed, and it is concluded that juxtaductal pulmonary artery coarctations should be specifically looked for before and during any type of surgical intervention in these patients.