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Virtual Pediatric Hospital: Paediapaedia: Scimitar Syndrome (Partially Anomalous Pulmonary Venous Return) (PAPVR) (Pulmonary Venolobar Syndrome) Paediapaedia: Cardiovascular Diseases

Scimitar Syndrome (Partially Anomalous Pulmonary Venous Return) (PAPVR) (Pulmonary Venolobar Syndrome)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Not applicable

Etiology/Pathophysiology:
A spectrum of defects in lung development associated with anomalies of the pulmonary vasculature and thorax that always includes hypoplasia and or aplasia of at least one lobe in the right lung.

Pathology:
Not applicable

Imaging Findings:
Hypoplasia/aplasia of greater than or equal to one lobe of the right lung manifests as decreased volume in the right hemithorax with shift of the mediastinum into the right hemithorax. There is usually partial anomalous pulmonary venous drainage to the inferior vena cava / portal vein / hepatic vein / right atrium, absent or decreased pulmonary arterial perfusion, an arterial supply coming from the thoracic or abdominal aorta or celiac axis, ipsilateral diaphragm defects, and thoracic defects. The scimitar comes from an anomalous pulmonary vein draining into the inferior vena cava either above or below the diaphragm.

DDX:

References:
See References Chapter.

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