Anomalies of great vessels associated with lung hypoplasia. The scimitar syndrome.

Abstract

IN THE PEDIATRIC age group, malformations of the pulmonary venous system often can produce characteristic roentgenographic features which, by themselves, are virtually diagnostic of the condition. Thus, total anomalous pulmonary venous drainage with connection to the left innominate vein by means of a vertical vein gives rise to the "figure of eight" or "snowman" configuration of the cardiac silhouette on frontal roentgenograms of the thorax.^1,2Total anomalous pulmonary venous drainage with connection below the diaphragm frequently yields the "ground glass" or "reticular" appearance of pulmonary venous obstruction in the peripheral lung fields on plain frontal roentgenograms of the thorax.³ Anomalous drainage involving connection of some or all of the pulmonary veins of the right lung to the inferior vena cava either below the diaphragm or at the junction of the inferior vena cava and the right atrium constitutes another roentgenographic and clinical entity. Halasz and associates⁴have pointed