Pulmonary Developmental Anomalies
PAPVR
Uwa Aideyan, MD
Peer Review Status: None
Forms:
- Associated with ASD
- Associated with hypogenetic lung (SCIMITAR syndrome)
Scimitar Syndrome
Almost exclusively right-sided
In almost all instances, the anomalous vein is a pulmonary
vein
It usually drains into the infradiaphragmatic IVC. Less
commonly drainage is to hepatic v., portal v., azygous v.,
coronary sinus or right atrium.
Occasionally, the PAPVR drains to the left atrium ("meandering
pulmonary vein")
In many cases of scimitar s., part of the right lung receives
arterial blood supply from the descending thoracic aorta or upper
abdominal aorta (systemic arterialization of the lung without
sequestration).
US, CT, MRI and angiography are useful for confirmation of the
PAPVR and determination of it's drainiage.
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