pulmonary venolobar (scimitar) syndrome

• always involves aplasia of one or more lobes of right lung

• variably involves:
  • partial anomalous pulmonary venous return (scimitar-shaped vein)
    • usually drains to IVC; but portal vein, hepatic vein, RA possible
  • absent or small pulmonary artery perfusing abnormal lung segment
    • arterial supply from thoracic/abdominal aorta or celiac axis
  • anomalies of hemidiaphragm on affected side
  • anomalies of bony thorax or thoracic soft tissues

© 2002, C.E. Kahn, Jr.
2002-04-18