Isolated scimitar vein is associated with a benign clinical course and requires no surgical intervention.1 Surgical repair is indicated in symptomatic cases or in those with pulmonary hypertension, increased pulmonary blood flow (Qp:Qs > 1.5), enlargement of the right cardiac chambers, stenosis of the scimitar vein, or associated anomalies that . Given the potential high incidence of postoperative occlusion, MRI provides hemodynamic and anatomic information for both initial and follow-up assessment of scimitar syndrome. (e-mail: kevinaneal@wustl.edu ). 2.

Anomalous pulmonary venous drainage (APVD) is the drainage of one or more pulmonary veins outside the left atrium. Scimitar syndrome is a rare congenital pulmonary anomaly characterized by a unilateral anomalous pulmonary venous drainage consisting of an aberrant vein that drains the lung blood flow into the systemic venous circulation (usually the inferior vena cava) and is associated with an ipsilateral pulmonary hypoplasia, underdevelopment of the ipsilateral pulmonary artery, and sometimes . The anomalous vein is curvilinear and parallels the right heart border to drain into the intra‐thoracic inferior vena cava. The syndrome associated with PAPVR is more commonly known as Scimitar . We report a case of a 40-year-old woman with scimitar syndrome discovered on chest X . A variant is described with the same roentgenographic appearance, but with drainage of the anomalous pulmonary vein into both the inferior vena cava and the left atrium; the atrial septum was intact.

72-1). Scimitar syndrome is a congenital heart disease characterized by an abnormal drainage of the right lung into the inferior vena cava, the right atrium or a variety of venous connections from the anomalous pulmonary vein to a systemic vein. A case is reported of a patient whose plain film radiographic findings showed a typical scimitar vein but whose echocardiography showed normal pulmonary venous drainage. Horseshoe lung is a rare congenital anomaly and mostly accompanied by scimitar syndrome. Scimitar Syndrome. Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. Posterior view of 3D MRI reconstruction of the cardiac anatomy, demonstrating the anomalous drainage of the scimitar vein into the right atrium/hepatic junction. Drainage into the hepatic vein, the portal vein or the left atrium can occur as an isolated lesion or together with other cardiac abnormalities like an atrial septal defect (ASD) . Findings with scimitar syndrome include a crescent-shaped anomalous pulmonary vein (resembling a Turkish sword or scimitar) paralleling the lower right heart border (Fig.
Imaging: With anomalous drainage of multiple veins, cardiomegaly with right heart enlargement and increased pulmonary flow are seen on chest radiography. The "scimitar sign" on plain chest radiograph is commonly attributed to the presence of a scimitar vein, which is a variant of partial anomalous pulmonary venous drainage. Generally, a part or all of the pulmonary veins of the lower lobe of the right lung and sometimes middle lobe veins drain into the vena cava inferior. No abstract listed. Congenital absence of a main branch of the pulmonary artery. Share. The "scimitar" sign is the unique radiologic finding of a "tubular-shaped opacity extending towards the diaphragm along the right side of the heart" [].This shape of the anomalous vein as it appears on imaging studies resembles a Turkish sword, the "Pala" []. However, in some cases, when the scimitar vein is masked by the overlying cardiac shadow or superadded lung pathology, Colour Doppler, computed tomography, and magnetic resonance imaging can be helpful by showing the abnormal vein and its insertion into the inferior vena cava. Scimitar Syndrome Bronchopulmonary Sequestration Vascular Malformations Syndrome Aortic Coarctation Heart Defects, Congenital Heart Diseases Hematoma Analytical, Diagnostic and Therapeutic Techniques and Equipment 7 The most constant and defining components of scimitar syndrome are hypogenetic lung and partial anomalous pulmonary venous return [3]. Scimitar vein plugging. Refers to the curved vascular shadow (resembling a short, curved Turkish sword called scimitar) representing an anomalous pulmonary vein draining the right lung that descends toward the diaphragm located to the right of the heart. Scimitar syndrome, also known as hypogenetic lung syndrome, is characterised by a hypoplastic lung that is drained by an anomalous pulmonary vein into the systemic venous system. Scimitar syndrome Imaging approaches M. Mearadji International Foundation for Pediatric Imaging Aid Rotterdam, The Netherlands . PMID:5078012 8. 26.1b). The scimitar syndrome is a constellation of cardiopulmonary abnormalities consisting of anomalous right pulmonary venous drainage of the right lung to the inferior caval vein, an anomalous systemic arterial blood supply from the abdominal aorta to the inferior segments of the right lung, variable degrees of hypoplasia of the right lung and pulmonary artery, dextroposition of the heart and . 3. Tortoriello TA, Vick III GW, Chung T, Bezold LI, Vincent JA. The chest roentgenograms showed displacement of the heart and mediastinum to the right with smaller right lung. Scimitar syndrome is a relatively uncommon association of congenital cardiopulmonary anomalies characterized by partial or complete anomalous pulmonary venous drainage from the right lung. Most aspects of this complex anomaly can be demonstrated via multidetector CT (MDCT). Radiological imaging of Scimitar Syndrome. Developed by renowned radiologists in each specialty, STATdx provides comprehensive decision support you can rely on - Scimitar Syndrome 1 Colour Doppler US image References A. Agayev (*) E. Yekeler The diagnosis of scimitar syndrome was confirmed and successfully repaired at operation. (7); alterna-tively, a meandering pulmonary vein may drain into both the left atrium and IVC (1, 13, 14). Scimitar syndrome, also known as hypogenetic lung syndrome, is characterized by a hypoplastic lung that is drained by an anomalous pulmonary vein into the systemic venous system. We report the imaging findings in a rare case of a unilateral single pulmonary vein identified on chest radiography and contrast-enhanced chest CT.Only a few cases have been reported in the literature describing this interesting rare anomaly that is often confused with arteriovenous malformation and hypogenetic lung syndrome (scimitar syndrome). The anomalous vein looks like the curved, Turkish sword (scimitar), hence the name. The adult form of scimitar syndrome is rare, and it is usually an incidental diagnosis based on the . N2 - We report a case of adult scimitar syndrome associated with Budd-Chiari syndrome in a young female. Biplane pul-monary angiography demonstrated the entire course of the scimitar vein with its drainage into the IVC in the venous phase (Figure9). In this paper, we present a case of a 15-day-old Saudi boy diagnosed with scimitar syndrome who presented with a chest infection and . Scimitar syndrome. The scimitar vein is demonstrated on MRI. Cardiac catheterization confirmed drainage of the scimitar vein to the left atrium and . 1. The British journal of radiology. Correction is indicated in adults with signs of pulmonary volume overload or right heart dilation. We present two baby girls who had horseshoe lung associated with right lung hypoplasia and scimitar vein.

W J HOLLIS. Can be isolated finding or combined with pulmonary hypoplasia (Scimitar syndrome). Scimitar syndrome describes a constellation of findings with the main finding being a PAPVR. Discussion. pulmonary vein'' by GOODMAN et al. From the Washington University School of Medicine in Saint Louis, Mallinckrodt Institute of Radiology, Campus Box 8131, 510 S Kingshighway Blvd, St Louis, MO 63110. Further evaluation with non-contrast computed tomography substantiated the finding of a partial anomalous venous drainage pattern and identified . The anomalous vein(s) may drain all or only a portion of the right lung and typically drains into the inferior vena cava. The scimitar sign is seen in partial anomalous pulmonary venous drainage of the right lower lobe. 717 The MRI study was performed on a 1.5-T scanner (Signa MR/i the scimitar vein (1.06 l/min, very similar to the difference in sys- Echo Speed, GE Medical Systems, Milwaukee, Wis.) using a torso temic-to-pulmonary artery flow). 10-year-old boy with bronchitis. The chest roentgenograms showed displacement of the heart and mediastinum to the right with smaller right lung. 3. Scimitar syndrome is a rare congenital abnormality resulting from right-sided pulmonary venous return to the inferior vena cava rather than to the left atrium. In "scimitar" syndrome, anomalously returning pulmonary veins, the place where the pulmonary veins are drained and the relation of the pulmonary veins with ASD are significant in terms of surgery. PMID:5641963 7. connection and tortuosity of the right lower lobe vein. Arterial supply from systemic artery was seen during surgery. INDEX TERM: Veins, pulmonary Radiology 113:601-603, December 1974 â ¢ ANOMALOUS pulmonary venous connection of .n. The scimitar syndrome is a rare but nevertheless important developmental cardiopulmonary abnormality of particular interest to the radiologist because it can be readily recognized on plain roentgenograms. Images: Scimitar vein pre- end post plugging. Abstract. Multi-detector computed tomography with 3D reconstruction allows clear depiction of vascular connections and associated anomalies, obviating the need for invasive procedures. A meandering pulmonary vein occurs when one of the pulmonary veins draining into the left atrium has an anomalous pulmonary . The x-ray also shows right lung hypoplasia (under-development) and the scimitar vein which reminds the curved Turkish sword that carries that name 53. Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium . Scimitar syndrome, also known as hypogenetic lung syndrome,is characterized by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. It is a type of partial anomalous pulmonary . A persistent left superior vena cava (PLSVC) is another thoracic venous anomaly. This left-to-right shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of recurrent pneumonia or significant . It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return. "Scimitar" is a descriptive term that refers to the curvilinear or arcuate course followed by the anomalous draining pulmonary vein, which has been likened to the shape of a Turkish sword called a scimitar.

The IVC is interrupted and drains via the posterior azygous. This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). These variations were confirmed by magnetic resonance imaging (MRI) and computed . Chest. The rare combination (3-5%) of an association of PAPVR, hypoplasia of the right lung and dextroposition of the heart is designated scimitar syndrome [2] . In the classic scimitar syndrome, a pulmonary vein draining all or part of the right lung enters the inferior vena cava. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome.
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