Scimitar Syndrome
2010; Lippincott Williams & Wilkins; Volume: 121; Issue: 23 Linguagem: Albanês
10.1161/circulationaha.109.931857
ISSN1524-4539
AutoresAlex Frydrychowicz, B. Landgraf, Oliver Wieben, Christopher J. François,
Tópico(s)Aerospace Engineering and Energy Systems
ResumoHomeCirculationVol. 121, No. 23Scimitar Syndrome Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBScimitar SyndromeAdded Value by Isotropic Flow-Sensitive Four-Dimensional Magnetic Resonance Imaging With PC-VIPR (Phase-Contrast Vastly Undersampled Isotropic Projection Reconstruction) Alex Frydrychowicz, MD, Ben Landgraf, BS, Oliver Wieben, PhD and Christopher J. François, MD Alex FrydrychowiczAlex Frydrychowicz From the Department of Radiology (A.F., B.L., C.J.F.) and Medical Physics (B.L., O.W.), University of Wisconsin–Madison, Wisconsin Institutes for Medical Research, Madison, Wis. , Ben LandgrafBen Landgraf From the Department of Radiology (A.F., B.L., C.J.F.) and Medical Physics (B.L., O.W.), University of Wisconsin–Madison, Wisconsin Institutes for Medical Research, Madison, Wis. , Oliver WiebenOliver Wieben From the Department of Radiology (A.F., B.L., C.J.F.) and Medical Physics (B.L., O.W.), University of Wisconsin–Madison, Wisconsin Institutes for Medical Research, Madison, Wis. and Christopher J. FrançoisChristopher J. François From the Department of Radiology (A.F., B.L., C.J.F.) and Medical Physics (B.L., O.W.), University of Wisconsin–Madison, Wisconsin Institutes for Medical Research, Madison, Wis. Originally published15 Jun 2010https://doi.org/10.1161/CIRCULATIONAHA.109.931857Circulation. 2010;121:e434–e436Scimitar, or pulmonary venolobar, syndrome is a rare but well-known congenital cardiovascular defect that includes a hypoplastic right pulmonary artery and right lung, which leads to displacement of cardiac structures into the right hemithorax, anomalous systemic arterial supply to the right lung, and a characteristically curved anomalous right pulmonary vein that drains into the inferior vena cava and resembles the curved Middle Eastern sword "scimitar."1,2 A variety of congenital thoracic abnormalities are associated with this specific type of partial anomalous pulmonary venous return.3Imaging, and specifically findings from magnetic resonance imaging, in an 18-month-old male (11 kg body weight) with known congenital right pulmonary venolobar syndrome with increasingly frequent cyanotic episodes are presented. Findings on chest radiography and contrast-enhanced computed tomography of the chest performed when the patient was 4 days old included right lung hypoplasia and partial anomalous pulmonary venous return with scimitar vein to the supradiaphragmatic inferior vena cava (Figure 1). Echocardiography identified the scimitar vein and an atrial septal defect. Cardiac magnetic resonance imaging, including 4-dimensional flow-sensitive magnetic resonance imaging, confirmed these findings but also identified additional cardiovascular abnormalities, including an additional partial anomalous pulmonary venous return from the upper right lung to the superior vena cava and an anomalous systemic artery from the upper abdominal aorta to the lower right lung (Figure 2). Further comprehensive analysis of flow, blood flow quantification, and detection of blood flow direction was feasible in all analyzed vessels. Download figureDownload PowerPointFigure 1. Chest radiograph (left) and chest computed tomography (right, A–D) in a 4-day-old boy with scimitar syndrome. Although right lung hypoplasia and shift of the mediastinal structures to the right are well delineated on the chest radiograph, the anomalous pulmonary venous return ("scimitar vein"; black arrowheads) cannot be readily appreciated. The scimitar vein is better appreciated with computed tomography (performed at age 4 days; white arrowheads), which also confirmed right lung hypoplasia and mild compression of the right lower lobe. Additional partial anomalous pulmonary venous return vessels were not identified, possibly owing to the small anatomic scale at that age and a lack of information on blood flow direction.Download figureDownload PowerPointFigure 2. A, Maximum-intensity projection of the phase-contrast angiogram in sagittal oblique direction as seen from 30° left anterior oblique view. In addition to the pronounced scimitar vein (ScimV), the hypoplastic right pulmonary artery (*), an additional partial anomalous pulmonary venous return vein in the right upper lobe (open white arrow), and the anomalous systemic artery from the celiac trunk to the right lower lung (white arrowheads) can be appreciated. B, Posterior view of segmented PC-VIPR angiography data with color-shaded surface display. For detailed understanding and ready apprehension, the oxygenized arterial (red), oxygenized partial anomalous pulmonary venous return (pink), deoxygenized venous and right ventricular (blue) structures, and portal venous system (yellow) were color-coded. SVC indicates superior vena cava; AAo, ascending aorta; LPA, left pulmonary artery; LA, left atrium; RA, right atrium; IVC, inferior vena cava; and DAo, descending aorta.With advanced magnetic resonance imaging approaches, simultaneous anatomic and functional hemodynamic imaging can be obtained by use of 4-dimensional flow-sensitive sequences such as PC-VIPR (phase-contrast vastly undersampled isotropic projection reconstruction).4 PC-VIPR magnetic resonance imaging was performed on a clinical 1.5T Signa HDx MR system (GE Medical Systems, Milwaukee, Wis) equipped with an 8-element phased-array cardiac coil and TwinSpeed gradient performance in "whole" mode (gradient strength=40 mT/m, maximum rise time=288 μs). Data were acquired during free breathing with respiratory gating. Parameters for the 4-dimensional flow sequence (PC-VIPR)4 were adapted to the specific anatomic demands: Echo time/repetition time=3.08/9 ms; flip angle=10°, bandwidth=62.5 kHz; velocity-encoding sensitivity=100 cm/s; field of view=256×256 mm; slab thickness=14 cm; 3-dimensional radial acquisitions with 256 data points in the readout direction; image volume=256×256×140 voxels; spatial resolution=1×1×1 mm3; and 12 time frames per cardiac cycle at a heart rate of 137 bpm. Offline image visualization was performed with Vitrea Advanced software (Vital Images Inc, Minnetonka, Minn) and MIMICS (Mimics Innovation Suite, Materialise, Ann Arbor, Mich) for image segmentation and morphology (Figure 2; online-only Data Supplement Movie I), which subsequently was used to specify regions for flow visualization and analysis with EnSight 9.0 (CEI, Apex, NC; Figure 3; online-only Data Supplement Movies I and II). Download figureDownload PowerPointFigure 3. Color-coded particle trace representation of blood flow contribution and hemodynamics in the right atrium from a posterior view. As opposed to previous descriptions, the blood flow in the right atrium showed overt changes in flow patterns, with a backward rotation of the superior vena cava inflow. Animated blood flow behavior can be appreciated in online-only Data Supplement Movie I and II. SVC indicates superior vena cava; IVC, inferior vena cava; ASD, atrial septal defect; ScimV, scimitar vein; and RA, right atrium.Four-dimensional flow imaging not only enables the analysis of cardiovascular morphology but also provides quantitative flow parameters and blood flow patterns all from a single acquisition, thereby aiding in the diagnosis, identification, and characterization of vessels. The derived high-resolution angiogram with isotropic spatial resolution depicts the altered cardiovascular anatomy with multiple partial anomalous pulmonary venous return veins and the hypoplastic right pulmonary artery (8 mm in diameter compared with 14 mm in the left pulmonary artery) in great detail without the need for intravenous contrast material (Figure 2; online-only Data Supplement Movie I). Furthermore, with these techniques, the velocity fields could be visualized and flow rates, directions, and volumes in any region of interest could be analyzed subsequent to the scan without the need for multiple 2-dimensional acquisitions. In this patient, flow quantification revealed a pulmonary-to-systemic flow ratio (QP/QS) of 1.33, with contribution of the scimitar vein to the inferior vena cava equal to 0.42 L/min and a left-to-right shunt through the atrial septal defect equal to 1.34 L/min. Four-dimensional flow furthermore depicted the various contributions to right atrial filling and mixture, which were less organized than described previously.5Without the need for an interventional procedure or multiple magnetic resonance acquisitions, in-depth visualization of hemodynamics by pathlines from the scimitar vein through the supradiaphragmatic inferior vena cava into the right atrium could be clearly separated from flow through the superior vena cava and atrial septal defect (Figure 3; online-only Data Supplement Movie II). Despite its thus far limited availability thus far and different clinical standards, including echocardiography, standard cardiovascular magnetic resonance imaging, computed tomography, and catheter angiography, the availability of anatomic and quantitative information from a single 5- to 10-minute acquisition could be especially suited for children with congenital cardiovascular abnormalities.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/121/23/e434/DC1.Source of FundingThe authors gratefully acknowledge funding from the National Heart, Lung, and Blood Institute (National Institutes of Health grant R01HL072260).DisclosuresNone.FootnotesCorrespondence to Alex Frydrychowicz, MD, University of Wisconsin School of Medicine and Public Health, Department of Radiology, 600 Highland Ave, CSC E1/322, Madison, WI 53729. E-mail [email protected]References1 Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage "scimitar syndrome." Bull Johns Hopkins Hosp. 1960; 107: 1–21.MedlineGoogle Scholar2 Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics. 1994; 14: 349–369.CrossrefMedlineGoogle Scholar3 Holt PD, Berdon WE, Marans Z, Griffiths S, Hsu D. Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome. Pediatr Radiol. 2004; 34: 409–413.CrossrefMedlineGoogle Scholar4 Gu T, Korosec FR, Block WF, Fain SB, Turk Q, Lum D, Zhou Y, Grist TM, Haughton V, Mistretta CA. PC VIPR: a high-speed 3D phase-contrast method for flow quantification and high-resolution angiography. AJNR Am J Neuroradiol. 2005; 26: 743–749.MedlineGoogle Scholar5 Kilner PJ, Yang GZ, Wilkes AJ, Mohiaddin RH, Firmin DN, Yacoub MH. Asymmetric redirection of flow through the heart. 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Millington S, Edwards S, Clark R, Dekker G, Arstall M and Ornaghi S (2021) The association between guidelines adherence and clinical outcomes during pregnancy in a cohort of women with cardiac co-morbidities, PLOS ONE, 10.1371/journal.pone.0255070, 16:7, (e0255070) Paidipati K, Kashyap R, Tadimeti H and Peyyeti M (2015) Incidentally Detected Mediastinal Vascular Anatomical Variants on PET-CT: A Pictorial Essay, Journal of Clinical Imaging Science, 10.4103/2156-7514.154353, 5, (19) Suzuki K, Takehara Y, Sakata M, Kawate M, Ohishi N, Sugiyama K, Akai T, Suzuki Y, Sugiyama M, Kawamura T, Morita Y, Kikuchi H, Hiramatsu Y, Yamamoto M, Nasu H, Johnson K, Wieben O, Kurachi K, Takeuchi H and Chalmers J (2021) Daikenchuto increases blood flow in the superior mesenteric artery in humans: A comparison study between four-dimensional phase-contrast vastly undersampled isotropic projection reconstruction magnetic resonance imaging and Doppler ultrasound, PLOS ONE, 10.1371/journal.pone.0245878, 16:1, (e0245878) June 15, 2010Vol 121, Issue 23 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.109.931857PMID: 20547935 Originally publishedJune 15, 2010 PDF download Advertisement SubjectsComputerized Tomography (CT)Congenital Heart DiseaseDevelopmental BiologyImaging
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