The Hypogenetic Lung (Scimitar) Syndrome
2019; Radiological Society of North America; Volume: 293; Issue: 3 Linguagem: Inglês
10.1148/radiol.2019191540
ISSN1527-1315
AutoresKevin Neal, Marilyn J. Siegel,
Tópico(s)Tracheal and airway disorders
ResumoHomeRadiologyVol. 293, No. 3 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyThe Hypogenetic Lung (Scimitar) SyndromeKevin A. Neal , Marilyn J. SiegelKevin A. Neal , Marilyn J. SiegelAuthor AffiliationsFrom the Washington University School of Medicine in Saint Louis, Mallinckrodt Institute of Radiology, Campus Box 8131, 510 S Kingshighway Blvd, St Louis, MO 63110.Address correspondence to K.A.N. (e-mail: [email protected]).Kevin A. Neal Marilyn J. SiegelPublished Online:Oct 29 2019https://doi.org/10.1148/radiol.2019191540MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractOnline supplemental material is available for this article.Download as PowerPointA 29-year-old woman was found to have hypogenetic lung syndrome (scimitar syndrome) after developing heart failure with severe pulmonary hypertension 3 years after pregnancy (pulmonary arterial pressures of 90/37 mm Hg [mean, 54 mm Hg], with oxygen saturation as measured with pulse oximetry of 100%). This subsequently improved with vasodilators (57/20 mm Hg; mean, 32 mm Hg). A right-to-left shunt was characterized by using phase-contrast cardiac MRI, with a pulmonary-to-systemic flow ratio of 1.8 and with 1.2 L/min flow in the “scimitar” vein. No other shunts were present.Adequate depiction of the ostium of the draining vein is key in planning surgical treatment (extracardiac vs intracardiac baffle). The cause of the scimitar syndrome is related to congenital right-sided pulmonary venous drainage with hypoplasia of the right lung, cardiac dextroposition, and systemic arterial supply to the right lower lobe (1). These are left-to-right shunts and can lead to pulmonary hypertension. If the shunt is large enough, right-sided heart failure may occur (Figure, Movie [online]).Anterior-oblique cinematic rendering from CT angiogram demonstrates a right-sided vertical pulmonary vein (the “scimitar”) (white arrow) extending to intrahepatic inferior vena cava (black arrow), representing partial anomalous pulmonary venous return. A hypertrophied feeding artery arises from the abdominal aorta and supplies portions of the right lower lobe (arrowhead).Download as PowerPointOpen in Image Viewer Get the Flash Player to see this video.Movie: Movie file of coronal cinematic rendering with 360° rotation.Download Original Video (11.8 MB)Disclosures of Conflicts of Interest: K.A.N. disclosed no relevant relationships. M.J.S. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: receives payment for lectures including service on speakers bureaus from Siemens Healthcare. Other relationships: disclosed no relevant relationships.Reference1. Lyen S, Wijesuriya S, Ngan-Soo E, et al. Anomalous pulmonary venous drainage: a pictorial essay with a CT focus. J Congenit Cardiol 2017;1(1):7. Crossref, Google ScholarArticle HistoryReceived: July 11 2019Revision requested: Aug 19 2019Revision received: Sept 4 2019Accepted: Sept 17 2019Published online: Oct 29 2019Published in print: Dec 2019 FiguresReferencesRelatedDetailsRecommended Articles Comprehensive Cross-sectional Imaging of the Pulmonary VeinsRadioGraphics2017Volume: 37Issue: 7pp. 1928-1954CT Appearance of Pulmonary Arteriovenous Malformations and MimicsRadioGraphics2022Volume: 42Issue: 1pp. 56-68Biatrial Drainage of the Right Superior Vena Cava: Imaging FindingsRadiology: Cardiothoracic Imaging2020Volume: 2Issue: 6Perfusion Scintigraphy in Diagnosis and Management of Thromboembolic Pulmonary HypertensionRadioGraphics2019Volume: 39Issue: 1pp. 169-185Imaging Manifestations and Interventional Treatments for Hereditary Hemorrhagic TelangiectasiaRadioGraphics2021Volume: 41Issue: 7pp. 2157-2175See More RSNA Education Exhibits Pulmonary Arteriovenous Malformations and MimicsDigital Posters2020Imaging of Congenital and Acquired Thoracic Venous Anomalies: What the Radiologist Needs to KnowDigital Posters2018Which Side Are You? Asymmetrical Congenital Anomalies of the Thoracic VesselsDigital Posters2019 RSNA Case Collection Partial anomalous pulmonary venous returnRSNA Case Collection2021Right upper PAPVR and persistent left SVCRSNA Case Collection2020Intrapulmonary Venous CollateralRSNA Case Collection2022 Vol. 293, No. 3 Metrics Altmetric Score PDF download
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