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‘PAPVR’

Scimitar syndrome

Author Ashley Davidoff MD

hypoplasia / aplasia of one or more lobes of lung

always on RIGHT

+/- partial APVR (to IVC, portal vein, hepatic vein.)

scimitar-shaped density is draining vein

  • Scimitar Syndrome
    01713b01 heart cardiac lung IVC inferior vena cava venous drainage pulmonary vein fx scimitar sign patrial anomalous venous return to IVC hypoplastic right lung dx congenital pulmonary venolobar syndrome CXR plain film chest Courtesy Ashley Davidoff MD
  • Scimitar Syndrome
    01714 heart cardiac lung IVC inferior vena cava venous drainage pulmonary vein fx scimitar sign patrial anomalous venous return to IVC hypoplastic right lung dx congenital pulmonary venolobar syndrome CXR plain film chest Courtesy Ashley Davidoff MD
  • one manifestation of a more generalized disorder called congenital pulmonary venolobar syndrome. This syndrome encompasses a number of congenital abnormalities including: hypogenetic lung, partial anomalous pulmonary venous return, absence of pulmonary arteries, pulmonary sequestration, absence of IVC, and accessory diaphragm. When hypogenetic lung and partial anomalous pulmonary venous return coexist, it is termed scimitar syndrome. The anomalous draining pulmonary vein most commonly drains into the IVC, but may on occasion drain into the hepatic veins, portal veins, azygous vein, coronary sinus, or right atrium. The abnormality is almost always right sided. Associated anomalies include branchial isomerism and anomalous arterial supply to the hypogenetic lung (usually systemic arteries such as bronchial).

    Web References

    Hawai Pediatric