The features of 11 cases of congenital pulmonary lymphangiectasis dying during the neonatal period are presented. Seven were associated with total anomalous pulmonary venous drainage with constriction of the common pulmonary vein at its junction with the general venous system. The anatomical features are compared with 14 infants with total anomalous pulmonary venous drainage without pulmonary lymphangiectasis.
Evidence from this series and from the published reports suggests that at least some examples of congenital pulmonary lymphangiectasis result from obstruction to the pulmonary venous return by anomalous pulmonary venous drainage or obstructive left heart anomalies. It is probable that congenital pulmonary lymphangiectasis will remain a bar to the successful surgical treatment in the newborn period of many patients with total anomalous pulmonary venous drainage.