Scimitar syndrome is a variant of partial anomalous pulmonary venous connection (PAPVC), in which all or part of the right lung is drained by right pulmonary veins that anomalously connect to the inferior vena cava (IVC). The affected lung and its associated airways are often hypoplastic. In addition, aortopulmonary collateral vessels may be involved on the affected side, causing sequestration ...

We report on a two-month-old infant with an unusual form of scimitar syndrome, associated with an absent right pulmonary artery, obstructed inferior vena cava, hemi-azygous continuation and the VACTERL association. The infant posed a major management problem and eventually died from a lower respiratory tract infection.

Scimitar syndrome (SS) is a rare congenital anomaly characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persistent left superior vena cava, and dextroposition of the heart. We report a rare variant of SS in a 44-year-old man ...

Scimitar Syndrome is a congenital condition resulting in anomalous pulmonary venous drainage from the lung into the inferior vena cava. It is often associated with cardiac and pulmonary abnormalities, both which are determinants in the severity and timing of presentation. True incidence of this condition is unknown and likely to be significantly underestimated as many patients remain asymptomat...