COARCTATION OF THE PULMONARY-ARTERY IN NEONATES - PREVALENCE, DIAGNOSIS, AND SURGICAL-TREATMENT

Stenosis or discontinuity of the central pulmonary artery at its bifur cation is often found in patients with congenital heart disease with i ntracardiac obstruction of the right side. The prevalence of this dise ase in autopsy cases is about 30%. The clinical prevalence has not bee n established vet because early diagnosis of this lesion is easily mis sed. Embryology suggests an etiologic similarity between coarctation o f the aorta and this type of stenosis: both are caused by invasion of ductal tissue into the respective great artery. Therefore, the term co arcation of the pulmonary artery is appropriate. To recognize or exclu de coarctation of the pulmonary artery, we examined left ventricular a ngiograms, aortograms, or both in the hepatoclavicular view in 25 cons ecutive neonates with severe arterial hypoxemia caused by right ventri cular 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 cas es was coarctation of the pulmonary artery present. Conversely, in 9 o f 17 neonates with a patent ductus arteriosus, coarctation of the pulm onary artery of different degree was demonstrated, even during prostag landin E1 infusion in 7 neonates. With regard to surgical procedures, ductal tissue in the pulmonary artery wall should be completely remove d. 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 preve nted.