What other congenital anomalies are often present?
Cardiac - e.g. ASD, patent ductus arteriosus (occurs in ~ 25%).
CT-scan confirms the abnormal pulmonary vein communicating with the inferior vena cava.
There is dextroposition of the heart and severe hypoplasia of the right pulmonary artery. The left pulmonary artery is enlarged.
On parenchymal windows, no fissure is clearly identified.