Cardiovascular ImagingHypogenetic lung syndrome
a group of
congenital anomalies consisting of hypoplasia of the right lung, hypoplasia of the right
pulmonary artery and partial anomalous
pulmonary connection of right
pulmonary veins to the inferior vena cava. The heart is displaced into the right hemithorax producing a
cardiac position called dextroposition. The right lung may have two rather than three lobes. This anomaly is also called
pulmonary venolobar syndrome, and the
scimitar syndrome. Chest
X-ray shows a small right lung, dextroposition of the heart, and the scimitar
vein. The scimitar
vein increases in diameter in its course towards the
diaphragm and its entry into the inferior vena cava.
CT and
MRI demonstrate the small right lung and
pulmonary artery and the course of the anomalous
pulmonary venous connection to the inferior vena cava.
CBH