1. List the various congenital anomalies associated with scimitar syndrome 2. Explain the role of CT and MRI to identify scimitar syndrome, its associations, and to quantify the extent of left-to-right shunt 3. Describe the differences between infantile manifestations of scimitar syndrome and manifestationsoccuringbeyond the early years of life

Scimitar syndrome is a rare congenital syndrome, occurring in 1-3 per 100,000 live births, for which the sine qua nonis anomalous venous drainage of the lung to the inferior vena cava (Fig. 2andFig. 3 ). Other names used to describe this syndrome arecongenital venolobar syndrome and hypogenetic lung syndrome.The embryology of scimitar syndrome is not well understood. An early disorder of lung bud development likely leads to the anomaly, although no single insult can explain all the associations. Radiologists are familiar with the scimitar sign,...

1. Review of scimitar syndrome 1.1 Definition Scimitar syndrome is a form of partial anomalous pulmonary venous connection, occurring nearly always on the right and associated primarily with: Ipsilateral lung and pulmonary artery hypoplasia Dextroposition of the heart Anomalous systemic arterial supply to the ipsilateral lung 1.2 Types Scimitar syndrome is divided into two types: Infantile Usually presents within the first few months of life Associated with congenital heart disease Complicated by congestive heart failure, pulmonary hypertension, or both Childhood/adult Degree of lung hypoplasia less...

Scimitar syndrome is more than a simple variant of partial anomalous pulmonary venous drainage. Anomalies of the ipsilateral lung and associated congenital cardiovascular and diaphragmatic malformations are frequentlypresent and should be recognized to optimize patient care.

Jeffrey P. Kanne, M.D. Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA Travis S. Henry, M.D. Department of Radiology, University of California at San Francisco, San Francisco, CA, USA Howard Mann, M.D. Department of Radiology, University of Utah, Salt Lake City, UT, USA Arlene Sirajuddin, M.D. National Heart, Lung, and Blood Institute,National Institutes of Health, Bethesda MD, USA J. David, Godwin, M.D. Department of Radiology, University of Washington, Seattle, WA, USA Authors are members of the Chest Radiologists...

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