Coarctation of the pulmonary artery in neonates. Prevalence, diagnosis, and surgical treatment.

Stenosis or discontinuity of the central pulmonary artery at its bifurcation is often found in patients with congenital heart disease with intracardiac obstruction of the right side. The prevalence of this disease in autopsy cases is about 30%. The clinical prevalence has not been established yet because early diagnosis of this lesion is easily missed. Embryology suggests an etiologic similarity between coarctation of the aorta and this type of stenosis: both are caused by invasion of ductal tissue into the respective great artery. Therefore, the term coarctation of the pulmonary artery is appropriate. To recognize or exclude coarctation of the pulmonary artery, we examined left ventricular angiograms, aortograms, or both in the hepato-clavicular++ view in 25 consecutive neonates with severe arterial hypoxemia caused by right ventricular outflow tract obstruction. The prevalence of coarctation of the pulmonary artery was 36% (9/25). Fifteen neonates had two ventricles, and the other 10 had different types of univentricular heart. In eight cases no patent ductus arteriosus was found, and in none of these cases was coarctation of the pulmonary artery present. Conversely, in 9 of 17 neonates with a patent ductus arteriosus, coarctation of the pulmonary artery of different degree was demonstrated, even during prostaglandin E1 infusion in 7 neonates. With regard to surgical procedures, ductal tissue in the pulmonary artery wall should be completely removed. The aim is to establish adequate blood flow to both right and left pulmonary arteries. In this way unilateral hypoplasia of the arterial tree and consecutive growth of tortuous bronchial collaterals is prevented.