Scimitar Syndrome DefinitionPage Contents1 Scimitar Syndrome Definition2 Scimitar Syndrome Synonyms3 Scimitar Syndrome Incidence4 Scimitar Syndrome Presentation5 Scimitar Syndrome Causes6 . (PDF) Scimitar syndrome: Incidence, treatment, and prognosis lung. There is a clear bimodal presentation of this syndrome with either an infantile manifestation or a pediatric/adult form. There were no mortalities and no incidence of pulmonary vein obstruction following their multipatch repair technique. (PDF) Surgery for scimitar syndrome: The Melbourne experience Medical Image of the Week: Scimitar Syndrome 3 Pulmonary venogram, left anterior oblique projection. REPAIR OF SCIMITAR SYNDROME 33 1 Chest radiograph illustrating the appearance and course of the anomalous right puhnonary, or scimitar, vein (arrow). The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. Scimitar syndrome is a rare congenital anomaly in which there is an anomalous pulmonary vein drain- ing most or all of the right lung into the inferior vena cava near the entrance to the atrium. Scimitar syndrome (SS) is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava. We report a case of a 40-year-old woman with scimitar syndrome discovered on chest X . Scimitar Syndrome. INTRODUCTION. Scimitar Syndrome. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. INTRODUCTION. 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. Read "Repair of Partial Anomalous Pulmonary Venous Connection in the Scimitar Syndrome Using VacuumAssisted Venous Drainage, Journal of Cardiac Surgery" on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. 4' 5 Dupuis and associates 6'7 identified two separate types of scimitar syndrome, the infantile and the adult Geggel and colleagues1 describe a multipatch repair technique for scimitar syndrome. 1 Introduction. Scimitar syndrome (SS) is an unusual condition of anomalous pulmonary venous return from the right lung to the inferior vena cava. Martinez SC, Eghtesady P, Bhalla S, Ludbrook PA (2015) Scimitar Syndrome: Multimodal Imaging Before and After Repair. Author information: (1)Department of Cardiovascular Surgery, Bordeaux Heart University Hospital, University of Bordeaux II, Bordeaux, France Tex Heart Inst JDec 42 . Scimitar syndrome is divided into 3 groups according to the age of the patients, as defined in a multicenter study involving 122 patients aged 1 to 58 years. 83 Rafael Meza et al. Scimitar syndrome was first described by Cooper in 1836. described for repair of scimitar syndrome that in the short to intermediate term has not been associated with postoperative pulmonary vein obstruction. Following repair, Scimitar vein stenosis occurs in approximately 20%. The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. In Scimitar syndrome, a curved-shaped anomalous pulmonary venous drainage is seen. The authors present a novel technique for intracardiac baffle in scimitar syndrome that reroutes scimitar vein drainage to the left atrium by approximating t. CiteSeerX - Document Details (Isaac Councill, Lee Giles, Pradeep Teregowda): Scimitar syndrome is a rare but serious congenital condition that consists of anomalous pulmonary venous drainage of the right lung to the inferior vena cava. - Infantile Scimitar Syndrome Repair Braz J Cardiovasc Surg 2021;36(1):133-6 Fig. We applied a variant of the atrial switch technique using autologous pericardial This anomalous pulmonary vein is referred to as the scimitar vein (SV). Scimitar syndrome is defined as anomalous pulmonary venous drainage from the right lung to the inferior vena cava (IVC). The atrial septum was extremely deviated to the left, and the left atrium was located entirely on the left of . A 26-year-old woman with partial anomalous pulmonary venous drainage into the right atrium (Scimitar syndrome) was successfully operated upon by incorporating an intraatrial conduit. Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. It is a variant of a partial anomalous pulmonary venous return that results in a left-to-right shunt, with a characteristic anatomic feature that resembles a backsword or a saber with a . Katherine Salciccioli, MD. The inci- The incidence in adults is reported to be 0.5-0.7%; classically, the adult form does not have pulmonary arterial hypertension, and may be unnoticed and asymptomatic for many years. Scimitar syndrome forms only 3% to 5% of all cases of partial anomalous pulmonary venous drainage. Without surgery to correct this left-to-right shunt, many patients are at risk of . Approximation of Scimitar Vein to Atrial Septal Defect During Scimitar Syndrome Repair By: Maxwell Kilcoyne, Chi Chi Do-Nguyen, Achintya Moulick, Vicki Mahan, Randy Stevens Kilcoyne MF, Do-Nguyen CC, Moulick A, Mahan V, Mesia CI, Stevens RM. The variant described in the case presented here, with total anomalous right pulmonary venous drainage into the superior and inferior vena cava, can be expected . The scimitar syndrome is a particular clinical problem characterized by a small right lung, resulting in the heart moving to the right (cardiac dextroposition), and an abnormal band shadow representing the abnormal venous drainage to the systemic veins (Fig. Vida and Guariento1 provide a timely, comprehensive review of pathophysiology, principles of diagnostic evaluation, and considerations for surgical repair for patients with scimitar syndrome. Scimitar syndrome forms only 3% to 5% of all cases of partial anomalous pulmonary venous drainage. The presence of scimitar syndrome is an indication for surgical repair, especially if associated ASD, pulmonary hypertension, pulmonary artery hypoplasia, or scimitar vein stenosis is present [10 . Group I comprises the adult form, which shows no pulmonary artery hypertension (PAH) and involves a small septal defect; it is the best tolerated, as was seen in one of our patients. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. See Editorial Commentary page e107. Thrombosis or stenosis of the scimitar vein is a serious complication of surgery. The hallmark feature is anomalous venous drainage of the right lung that produces a distinctive image on anteroposterior chest roentgenogram (Fig 1 . Postoperative pulmonary venous obstruction is prevalent, especially in the infants. The infantile form presents early in life with symptoms of tachypnea, chest infection, heart failure, and failure to thrive, and, as a result, it always requires intervention [ 1 ] . Successful surgical repair of the syndrome was first reported by Kirklin, Ellis, and Wood 3 in 1956. The association with other congenital defects, 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. Considering the . . Overview and natural history. BACKGROUND:Scimitar syndrome (SCS) is a rare congenital cardiopulmonary malformation, characterized by anomalous pulmonary venous drainage from the right lung associated with aortopulmonary collateral arteries and pulmonary hypoplasia. Scimitar syndrome is a rare congenital cardiac anomaly that represents 0.06% of the congenital cardiac population [].This syndrome is characterized by partially anomalous pulmonary venous drainage of typically the entire right lung, but may occasionally drain the lower or middle lobe [].The vein usually descends vertically anterior to the lung hilum and enters the inferior vena . Subclavian flap repair is a common surgical technique used, where the origin and proximal left subclavian artery is excised . Up to 25% of cases are associated with other heart diseases. Scimitar syndrome is a complex constellation of vascular, bronchial, and parenchymal malformations of the right lung. 2 The syndrome is almost invariably associated with other cardiac and extracardiac . Scimitar syndrome describes a specific subset of partial anomalous pulmonary venous return (PAPVR) in which an anomalous right sided pulmonary vein(s) drains either above or below the diaphragm into the inferior vena cava. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Overview and natural history. These include occlusion of the systemic arterial collaterals, right pneumonectomy, and complete repair. Dupuis and associates6, 7 identified two separate types of scimitar syndrome, the infantile and the adult forms. Scimitar syndrome. Pulmonary artery hypertension and hemoptysis both are uncommon features of this syndrome in adult life. CONCLUSION: We conclude that age at detection of scimitar syndrome is important in predicting outcome. Case description. Scimitar Syndrome is a rare congenital heart defect. The Journal of Thoracic and Cardiovascular Surgery c Volume 152 . Surgical repair is indicated in symptomatic patients, with often good results. It is usually detected in early childhood with symptoms of recurrent chest infection and finding of pulmonary hypertension due to left to right shunt. Surgical repair involves re-implantation of the anomalous vein into the left atrium and can be a complex and difficult procedure, however it can be accomplished with low morbidity and mortality at specialized centers (3).
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