Scimitar Sign Research Articles

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment

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, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as “the scimitar sign”, is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.

Imperforate anus associated with anomalous pulmonary venous return in scimitar syndrome. Case report from a tertiary hospital in Ethiopia

BackgroundScimitar syndrome is a rare form of partial anomalous pulmonary venous drainage associated with pulmonary hypertension and congestive heart failure that may lead to death in the newborn infant. Although it is described with anomalies of the lung, heart and their vascular structure, extremely rare association with imperforate anus had been reported. The third case of Scimitar syndrome and imperforate anus will be reported in this case report.Case presentationA 3 days old male neonate with imperforate anus presented with abdominal distention. Loop colostomy was done to relieve abdominal distension.The chest x-ray revealed a curved shadow on the right mid lung zone extending to the diaphragm abutting and indenting the inferior vena cava (scimitar sign).Abdominal ultrasound, transthoracic echocardiography and computerized tomographic angiography confirmed the presence of Scimitar vein and associated dextro-position of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, secundum atrial septal defect with bidirectional shunt, patent ductus arteriosus, pulmonary hypertension, left superior vena cava, and systemic collateral arteries feeding the lower lobe of the right lung.The rare association of scimitar syndrome with imperforate anus is discussed.ConclusionScimitar syndrome associated with imperforate anus with and without VACTERL association has been reported previously only in four cases. The knowledge of association between imperforate anus and Scimitar syndrome helps for early detection and management of cases. It is recommended to have high index of suspicion in every newborn with imperforate anus to check for symptoms of dextro-position of the heart, right lung hypoplasia which may be indicate scimitar syndrome.

Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins.

We aimed to review computed tomography and magnetic resonance angiography of congenital anomalies of pulmonary veins. Total anomalous pulmonary venous return shows all pulmonary veins drain abnormally in another site rather than left atrium. Imaging can detect anomalous veins either supracardiac, infracardiac, or mixed. Partial anomalous pulmonary venous return shows some pulmonary vein have abnormal drainage that well delineated with computed tomography angiography. Scimitar syndrome is a type of partial anomalous pulmonary venous return where the pulmonary veins of the right lung drain infracardiac and is associated with right lung hypoplasia and dextrocardia. Pseudoscimitar show anomalous vein that takes a tortuous course and drains into the left atrium producing a false-positive scimitar sign. Cor triatriatum shows septum divide left atrium with proximal chamber receives blood flow from the pulmonary veins. Levoatriocardinal vein is an anomalous connection between the left atrium and anomalous vein from systemic venous system that is embryo logically derived from the cardinal veins. Computed tomography angiography can detect pulmonary vein stenosis, atresia, hypoplasia, and varix. Imaging is important for intimal diagnosis and detects the anomalous vessels and its connection, presence of stenosis, and associated other congenital cardiac anomalies. Also, it is a great role in assessment of patients after surgery.

Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report.

BackgroundScimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel.Case summaryA 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the ‘scimitar sign’. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign.DiscussionAlthough the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.

Scimitar Syndrome: An Incidental Finding in an Older Adult

Scimitar syndrome is a rare partial anomalous pulmonary venous connection, resulting in partial or complete drainage of oxygenated blood into the systemic venous circulation via the inferior vena cava. Functionally, a left-to-right shunt ensues which can be of varying physiological consequence. We present the case of a 71-year-old gentleman who presented in extremis with hypercapneic respiratory failure due to an infective exacerbation of chronic obstructive airways disease. Initial electrocardiograms demonstrated ST depression in the apical leads with significant bradycardia resembling a junctional rhythm. Serial troponins were 28 ng/L and 957 ng/L, respectively, confirming the diagnosis of a non-ST segment elevation myocardial infarction. Coronary angiography revealed occlusive triple vessel disease, thus he was transferred for coronary artery bypass grafting. Preoperative review of his chest X-ray demonstrated scimitar sign and hypoinflation of the right lung. Cardiac magnetic resonance imaging confirmed partial anomalous pulmonary venous return with significant shunt detected (Qp:Qs = 2.0). Owing to his age, no correction of his partial anomalous pulmonary venous return was undertaken at the time of coronary artery bypass grafting. The scimitar vein is an uncommon anatomical variation often detected incidentally on plain chest radiography. This case demonstrates that although individuals may be asymptomatic at baseline, the associated left-to-right shunt may contribute to significant cardiorespiratory decompensation in the presence of additional insults, such as underlying pulmonary disease or an acute coronary syndrome. A thorough preoperative work-up confirming significance of the shunt and the specific structural anomalies present is essential prior to thoracic interventions, from both the perspective of surgical planning and anaesthesic management.

Case 2/2016 - Scimitar Sign with Right Pulmonary Vein Drainage into the Right Atrium

Clinical data: the characteristic signs of the scimitar syndrome with right pulmonary hypoplasia were discovered in an asymptomatic patient through routine chest X-ray, in the presence of dengue. On physical examination, the patient was in good general health status, eupneic, normal skin color, with normal pulses. His weight was 54 kg, height 155 cm, blood pressure of 100/60 mmHg, heart rate of 88 bpm. The aorta was not palpable at the sternal notch. In the precordium, there were mild impulses at the left and right sternal borders and the apex beat was not palpable. Heart sounds were normal, with constant splitting of the second heart sound, with discreet and rough ejection systolic murmur in the pulmonary area. The liver was not palpable and in the lungs, breath sounds were less audible in the right lower pulmonary lobe.

Scimitar Syndrome a Rare Cause of Isolated Esophgeal Varices

We present a rare case of isolated esophageal varices arising from Scimitar syndrome, an anomalous connection of the right pulmonary vein to the Inferior vena cava (IVC). A 53 year old female with past medical history of Scimitar syndrome repaired at age 20, with dissection of the anomalous right pulmonary vein and connecting it to the right atrium, and then creating an intra-atrial pericardial baffle directing venous flow to the left atrium presented with epigastric pain. She denied history of gastrointestinal bleeding or chest pain. Vital signs and exam were normal. Labs were normal. CT Chest, abdomen and pelvis showed mild mediastinal enlargement, a small right pulmonary artery, loss of right lung volume and diffuse distal esophageal wall prominence. The liver, spleen & pancreas were unremarkable. For epigastric pain, she underwent an EGD showing H.pylori gastritis and grade III esophageal varices in the mid and distal esophagus. She was treated for H. pylori gastritis. Endoscopic US showed esophageal varices, a lymph node in the aortopulmonary region and normal flow in the portal and splenic veins. For further workup of her esophageal varices in the setting of Scimitar syndrome and in the absence of portal hypertension, she underwent a cardiac catheterization. The cardiac catheterization showed normal right atrial and left ventricular end diastolic pressures. Also right pulmonary venous hypertension was noted, with the pulmonary venous collaterals draining into the azygous venous system, intercostal veins and then into the para esophageal venous system producing esophageal varices. Blood flow in the baffle from the right lower pulmonary vein into left atrium was not visualized, suggestive of obstruction of the surgical baffle. The study concluded that the right pulmonary venous flow drained into the systemic circulation via the paraesophageal veins. Scimitar syndrome aka pulmonary venolobar syndrome is a rare congenital heart malformation, with a prevalence of 0.5-1%. It is characterized by partial or complete anomalous drainage of the right pulmonary vein into the IVC, right lung hypoplasia and mediastinal shift. The ‘Scimitar sign' is a curvilinear shadow resembling a curved Turkish sword ‘scimitar' created by an abnormal right pulmonary vein draining into the IVC. The common causes of esophageal varices in an adult are portal vein thrombosis, liver cirrhosis, SVC obstruction, mediastinal tumors, thymoma or chronic fibrosing mediastinitis. Esophageal varices arising from an anomalous pulmonary venous return is very rare. In this case, obstruction of the surgical baffle resulted in esophageal varices without portal hypertension. Patient was referred to surgery for evaluation of right lower lobe pneumonectomy or creation of another surgical baffle which may diminish the esophgeal varices.Figure 1Figure 2Figure 3

An unusual cause of scimitar sign: Anomalous unilateral single right pulmonary vein associated with accessory liver lobe

We report a case of an anomalous unilateral single right pulmonary vein resembling a scimitar sign on chest X-ray. 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). This report briefly discusses the embryology of the pulmonary veins, classification of the pulmonary venous anomalies, and various causes of scimitar sign.

Anterior sacral Meningocele Associated with Tethered Cord Syndrome Presenting with Constipation: US, CT, MRI and X-Ray Findings

Anterior sacral meningocele is characterized by the herniation of the thecal sac into the retroperitoneal space. A 33-year-old man was referred to our hospital with the complaints of a longlasting constipation and nonspecific lower abdominal pain. At ultrasound, a cystic mass was detected at the presacral space. Computed Tomography revealed a defect at the anterior aspect of the sacrum, together with a presacral cystic mass which showed a connection with the dural sac. At Magnetic Resonance Imaging, it was demonstrated that the cystic mass made an impression on the rectum, and did not possess any solid components. An anteroposterior pelvis radiogram demonstrated a sacral deformity and the scimitar sign. Upon these imaging findings, the patient got the diagnosis of an anterior sacral meningocele. The possibility of an anterior sacral meningocele must always be kept in mind in the differential diagnosis of a pelvic cystic mass.

A rare case of isolated partial anomalous pulmonary venous connection to the inferior vena cava.

IMAGES IN MEDICINE A 34-year-old male came to our hospital for a routine master health check up. He was apparently normal and had no significant history. His chest X-ray postero anterior (PA) view showed a curvilinear radioopaque shadow in the right, mid and lower paracardiac location, coursing towards the diaphragm [Table/Fig-1]. A preliminary diagnosis of scimitar sign was made and a CT pulmonary angiogram was performed to confirm the diagnosis and to identify the extent of the vascular anomaly and any associated anomaly. It revealed an anomalous right superior pulmonary vein draining into the inferior vena cava [Table/Fig-2]. All the other pulmonary veins were draining normally into the left atrium. The pulmonary arteries were normal. There was no associated lung hypoplasia, sequestration or any R ad io lo g y S ec tio n A Rare Case of Isolated Partial Anomalous Pulmonary Venous Connection to the Inferior Vena Cava

Scimitar Syndrome: The Role of Cardiac Magnetic Resonance

A 35-year-old woman with no relevant medical history presented with dyspnea on moderate exertion. Physical examination detected a mild mid-systolic murmur, and the electrocardiogram showed negative T waves in the right precordial leads. On chest radiography, the right lung was mildly hypoplastic and showed a linear opacity (Figure 1A, arrow), corresponding to a vascular structure (scimitar sign). Transthoracic echocardiography showed dilation of the right chambers with no pulmonary hypertension, and venous flow entering the inferior vena cava (Figures 1B–D). The transesophageal echocardiogram additionally revealed an ostium secundum atrial septal defect with a left-to-right shunt (Figure 1E). Magnetic resonance imaging confirmed the diagnosis of a large supradiaphragmatic right pulmonary vein draining into the left inferior vena cava adjacent to the right atrium (Figures 1F and 2A, arrow). Based on the patient’s symptoms and the significant systemic-pulmonary shunt (Qp/Qs, 1.9:1.0), a decision was made to perform corrective surgery of the partial anomalous pulmonary venous drainage. The atrial septal defect was widened and a polytetrafluoroethylene tube was placed within the right atrium, redirecting flow from the inferior vena cava to the left atrium through the defect. The patient was discharged 7 days postprocedure, with no complications. At 3 weeks, cardiac magnetic resonance showed that the tube was patent (Figure 2B, asterisk) and the size of the right ventricle had returned to normal. At 6 months, the patient was asymptomatic. In this case, cardiac magnetic resonance imaging enabled confirmation of the diagnosis of partial anomalous pulmonary drainage, selection of the best surgical technique, and precise patient follow-up. Figure 1. Figure 2.

症例 "Scimitar sign"を呈しscimitar症候群と鑑別を要した1例

症例 "Scimitar sign"を呈しscimitar症候群と鑑別を要した1例

A 21 Years Old Woman, with Chest Pain and Cardiac Dextroposition Pulmonary Hypoplasia or Scimitar Syndrome?

Background : Physicians seldom encounter chest x-ray of adult patients diagnosed with dextrocardia. Case presentation: Here, we present the case of a 21-year-old woman who referred to the hospital with the clinical diagnosis of dextrocardia but, she has pulmonary hypoplasia and this rare disease in adults may be a component of scimitar syndrome and scimitar sign is not pathognomonic to define the syndrome. MR angiography was done, but finally the diagnosis was hypoplasia of right upper lobe and aplasia of middle lobe. Conclusion: As lung hypoplasia is rare in adults, some cases are misdiagnosed as dextrocardia on chest x-ray.

Incidental meandering right pulmonary vein, literature review and proposed nomenclature revision

We report a case of an anomalous pulmonary vein on chest X-ray resembling a scimitar sign in an 80-year-old female undergoing investigation of syncope. Multislice computed tomography (CT) with multiplanar reformatting and maximum intensity projections demonstrated an aberrant right inferior pulmonary vein coursing inferomedially towards the diaphragm before turning superiorly and draining normally into the left atrium. The diagnosis of an incidental meandering right pulmonary vein was established. The case is used to review the literature on this rare pulmonary anomaly, including pathogenesis, its relationship with scimitar syndrome and scimitar variant, and diagnosis, with an emphasis on the role modern CT techniques can play in non-invasive diagnosis. A revision to the nomenclature of pulmonary vascular anomalies is proposed to help reduce confusion in the literature.

Anomalous unilateral single pulmonary vein versus scimitar syndrome: Comparison of two paediatric cases and a review of the literature

The 'scimitar sign' on plain chest radiograph is most commonly attributed to the presence of a scimitar vein, which is a variant of partial anomalous pulmonary venous drainage (PAPVD). An anomalous unilateral single pulmonary vein (AUSPV) is a very rare anomaly, albeit benign, which can also present with the scimitar sign. Despite a similar radiographic appearance, these two anomalies have very different prognostic implications. Moreover, differentiation between the two is important in the paediatric population to prevent unnecessary investigations. We present two paediatric patients who presented with the scimitar sign on plain chest radiograph, with one having PAPVD and the other having AUSPV, and provide a review of the literature on AUSPV, an anomaly which most likely shares a similar pathogenesis to the scimitar vein of PAPVD.

A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.

Meandering Inferior Vena Cava in a Child With Pseudo-Scimitar Syndrome

An 11-year-old boy underwent chest roentgenogram for symptoms of pneumonia and was found to have dextroposed heart with a scimitar sign (Fig 1, white arrow). An echocardiogram was inconclusive regarding pulmonary venous drainage. A cardiac magnetic resonance image revealed dextroposition of the heart, hypoplastic right lung and right pulmonary artery, right lobar sequestration, eventration of the right hemidiaphragm, and normal pulmonary venous drainage. Ab-

Total Anomalous Pulmonary Venous Return with connection to the Supradiaphragmatic Inferior Vena Cava: Assessment and Diagnosis by Multidetector Computed Tomography.

ig. 1. The frontal chest radiograph showing the anomalous vascular tructure coursing to the right cardiophrenic angle (“scimitar sign”). below the diaphragmatic shape had also been reported (“scimitar sign”). Contrast-enhanced multidetector-row computed tomography (MDCT) was performed (Fig. 2a and b). The MDCT examination confirmed the dilatation of right heart chambers and the increased size of the pulmonary arteries. An anomalous pulmonary vein return draining the entire right lung into the inferior vena cava was also demonstrated. The normal main pulmonary veins of the right lung connecting to the left atrium were not shown (total return). The supradiaphragmatic vena cava was enlarged and showed early filling with contrast medium. The diagnosis of total anomalous pulmonary venous return was made and the patient was referred to surgical evaluation. Anomalous pulmonary venous returns (APVRs) are rare and complex congenital malformations that lead to left-to-right blood shunt and pulmonary hypertension [1–5]. When diagnosed in adults they manifest with respiratory insufficiency and recurrent pulmonary infections. Chest radiograph may be indicative of the

Scimitar syndrome

A 38-year-old-female presented to our unit for investigation of an incidental diagnosis of right ventricular dilatation. Transthoracic colour Doppler echocardiography demonstrated an anomalous flow into the inferior vena cava. The suspicion of partial anomalous venous drainage was supported by the finding of a scimitar sign on chest X-ray. Left and right heart catheterization demonstrated a significant left-to-right shunt (Qp/Qs = 2.22) with a step-up in oxygen saturation from the inferior vena cava (IVC) to lower right atrium. Selective angiography confirmed drainage of an anomalous pulmonary vein to the IVC-atrial junction. Scimitar syndrome is a rare vascular anomaly that can manifest in childhood with signs of right heart failure and/or recurrent pneumonia. When asymptomatic, this anomaly can be accidentally discovered in adulthood and should be suspected in cases of left-to-right shunt without evidence of obvious cardiac structural defects.

Scimitar Sign in a Patient With an Atrial Septal Defect: A Comprehensive Noninvasive Assessment With Transthoracic Echocardiography and Computed Tomography

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4] A 30-year-old woman was referred for a percutaneous atrial septal defect (ASD) closure. She was acyanotic. She had a systolic ejection murmur and a wide-fixed split S2. The chest radiograph showed cardiomegaly; pulmonary