Scimitar Syndrome
2008; Lippincott Williams & Wilkins; Volume: 118; Issue: 3 Linguagem: Albanês
10.1161/circulationaha.107.748707
ISSN1524-4539
AutoresEmanuele Gavazzi, Marco Ravanelli, Davide Farina, Maria Elena Chiari, Roberto Maroldi,
Tópico(s)Congenital Heart Disease Studies
ResumoHomeCirculationVol. 118, No. 3Scimitar Syndrome Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBScimitar SyndromeComprehensive, Noninvasive Assessment With Cardiovascular Magnetic Resonance Imaging Emanuele Gavazzi, MD, Marco Ravanelli, MD, Davide Farina, MD, Maria Elena Chiari, MD and Roberto Maroldi, MD Emanuele GavazziEmanuele Gavazzi From Department of Radiology, University of Brescia, Italy. , Marco RavanelliMarco Ravanelli From Department of Radiology, University of Brescia, Italy. , Davide FarinaDavide Farina From Department of Radiology, University of Brescia, Italy. , Maria Elena ChiariMaria Elena Chiari From Department of Radiology, University of Brescia, Italy. and Roberto MaroldiRoberto Maroldi From Department of Radiology, University of Brescia, Italy. Originally published15 Jul 2008https://doi.org/10.1161/CIRCULATIONAHA.107.748707Circulation. 2008;118:e63–e64Scimitar syndrome is a rare vascular anomaly whereby a partial anomalous pulmonary venous drainage to the inferior vena cava results in left-to-right shunt. Scimitar syndrome can be associated with congenital cardiovascular defects (dextrocardia, atrial septal defects, and right pulmonary artery hypoplasia), pulmonary anomalies (hypoplasia, sequestration), and tracheobronchial anomalies. Thus, it can manifest with heart failure and recurrent pneumonia.1,2Diagnosis is obtained during childhood, when symptoms related to relevant shunt and other cardiovascular and pulmonary anomalies are present. When asymptomatic, scimitar syndrome can be accidentally discovered in adulthood.3Standard chest x-ray evaluation is usually pathognomonic (Figure 1), although it is mandatory that a cross-sectional imaging technique be used to confirm the diagnosis and to obtain comprehensive assessment of any associated anomalies. Download figureDownload PowerPointFigure 1. A 36-year-old, healthy woman with an unremarkable past clinical history and normal ECG. Chest x-ray imaging, performed before minor maxillofacial surgery, shows linear opacity in the right lung (arrows) consistent with a vascular structure (a scimitar sign).Both computed tomography and magnetic resonance imaging (MRI) allow accurate diagnosis. In our experience, MRI is preferred because it allows the patient to avoid exposure to ionizing radiation and it does not require administration of contrast agent. Fast imaging with steady-state precession (TrueFISP) bright blood sequences in standard planes (Figure 2) provides excellent anatomic visualization; cine magnetic resonance sequences (balanced steady-state free precession technique) allow fast and reliable assessment of cardiac function and excellent depiction of associated cardiac anomalies; and flow maps acquired by means of phase-contrast sequences permit the demonstration of flow within the anomalous vessel as well as precise quantification of the left-to-right shunt (Figure 3 and Data Supplement Movie). Download figureDownload PowerPointFigure 2. Multislice, single-phase, fast imaging with steady-state precession (TrueFISP) sequence on coronal (A and B) and axial (C and D) planes shows an anomalous vessel in the right lung (arrows) connected to the inferior vena cava.Download figureDownload PowerPointFigure 3. Phase-contrast sequence (A) shows that flow within the anomalous vessel (arrow) has the same craniocaudal direction as flow within descending aorta (arrowhead). Analysis of the flow curve in the anomalous vessel (B). Values are negative because flow was encoded in the caudocranial direction (peak velocity 42 cm/s, average flow 1.3 L/min).In the present case, we obtained chest x-ray images of a 36-year-old woman before she underwent minor maxillofacial surgery (Figure 1). Her past clinical history was unremarkable, and ECG results were normal. On the basis of MRI findings, the final diagnosis of scimitar syndrome was made (Figures 2 and 3 and Movie). The patient was asymptomatic, and the pulmonary-to-systemic blood flow ratio was 1.14; thus surgery was not indicated. Nonetheless, because cine MRI showed slight increases in right ventricle volume (telediastolic 111 mL/m2, telesystolic 60 mL/m2) the patient will be followed clinically and echocardiographically.In conclusion, we think cardiovascular MRI without administration of contrast medium is the preferred technique for diagnosing scimitar syndrome, as it allows for comprehensive assessment of the anomalous vessel and heart and thus provides sufficient information for appropriate surgical or conservative management.The online-only Data Supplement, which contains a movie, can be found at http://circ.ahajournals.org/cgi/content/full/118/3/e63/DC1.DisclosuresNone.FootnotesCorrespondence to D. Farina, MD, Department of Radiology, University of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy. E-mail [email protected]References1 De Lucas EM, Canga A, Sadaba P, Martin-Duran R, Otero M, Cerezal L. Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity encoded cine MRI. Pediatr Radiol. 2003; 33: 716–718.CrossrefMedlineGoogle Scholar2 Kramer U, Dörnberger V, Fenchel M, Stauder N, Claussen CD, Miller S. Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol. 2003; 13: L147–L150.CrossrefMedlineGoogle Scholar3 Khan MA, Torres AJ, Printz BF, Prakash A. Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy. Am J Cardiol. 2005; 96: 1313–1316.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By D'Angelo T, Blandino A, Saitta M, Agati S, Romeo P and Mazziotti S (2021) A Rare Variant of Hypogenetic Lung Syndrome Mimicking Scimitar Vein, The Annals of Thoracic Surgery, 10.1016/j.athoracsur.2021.01.020, 112:3, (e173-e176), Online publication date: 1-Sep-2021. Jackson N, Nokes B, Sakata K, Cummings K and Vaszar L (2019) An unusual variant of scimitar syndrome predisposing to recurrent pneumonia, Respiratory Medicine Case Reports, 10.1016/j.rmcr.2019.01.010, 26, (240-243), . (2017) Scimitar Syndrome Diagnostic Imaging: Pediatrics, 10.1016/B978-0-323-44306-7.50103-1, (288-289), . Chan D, Skillington P and Laufer E (2015) Adult Scimitar Syndrome: A Surgical Approach, Heart, Lung and Circulation, 10.1016/j.hlc.2015.01.020, 24:7, (e115-e117), Online publication date: 1-Jul-2015. Ormeci T and Gunluoglu M (2014) Scimitar syndrome associated with gallbladder duplication, Clinical Imaging, 10.1016/j.clinimag.2013.12.015, 38:3, (346-349), Online publication date: 1-May-2014. Blondiaux E, Dacher J, Vivier P, Sirinelli D and Ducou le Pointe H (2012) Imagerie des retours veineux systémiques et pulmonaires, EMC - Radiologie et imagerie médicale - Cardiovasculaire - Thoracique - Cervicale, 10.1016/S1879-8535(12)58241-3, 7:4, (1-14), Online publication date: 1-Nov-2012. Meaney J and Beddy P (2012) Pulmonary MRA Magnetic Resonance Angiography, 10.1007/978-1-4419-1686-0_19, (253-268), . Ramirez-Marrero M and de Mora-Martin M (2012) Scimitar Syndrome in an Asymptomatic Adult: Fortuitous Diagnosis by Imaging Technique, Case Reports in Vascular Medicine, 10.1155/2012/138541, 2012, (1-3), . Martinez-Jimenez S, Heyneman L, McAdams H, Jasinowodolinski D, Rossi S, Restrepo C and Washington L (2010) Nonsurgical Extracardiac Vascular Shunts in the Thorax: Clinical and Imaging Characteristics, RadioGraphics, 10.1148/rg.e41, 30:5, (e41), Online publication date: 1-Sep-2010. Wong D, Grosse-Wortmann L and Yoo S (2010) Transpleural Pulmonary-to-Systemic Venous Collaterals in a Case With Obstructed Scimitar Vein, Pediatric Cardiology, 10.1007/s00246-010-9741-6, 31:7, (1086-1088), Online publication date: 1-Oct-2010. Yehia B, Bachmann J and Traill T (2010) Scimitar Syndrome: A Rare Cause of Dyspnea in Adults, Southern Medical Journal, 10.1097/SMJ.0b013e3181dfd59e, 103:6, (578-580), Online publication date: 1-Jun-2010. Vida V, Speggiorin S, Padalino M, Crupi G, Marcelletti C, Zannini L, Frigiola A, Varrica A, Di Carlo D, Di Donato R, Murzi B, Bernabei M, Boccuzzo G and Stellin G (2009) The Scimitar Syndrome: An Italian Multicenter Study, The Annals of Thoracic Surgery, 10.1016/j.athoracsur.2009.04.099, 88:2, (440-444), Online publication date: 1-Aug-2009. July 15, 2008Vol 118, Issue 3 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.107.748707PMID: 18625898 Originally publishedJuly 15, 2008 PDF download Advertisement SubjectsComputerized Tomography (CT)Imaging
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