PDF Successful Repair of Infantile Scimitar Syndrome with ... Scimitar syndrome was first described by Cooper in 1836. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Scimitar Syndrome. Scimitar syndrome with atrial fibrillation: Repair in an adult Scimitar Syndrome is a rare congenital heart defect. The scimitar syndrome is a rare congenital anomaly that needed to be diagnosed early and taken care of in time. This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The patient was diagnosed with Scimitar Syndrome, a rare congenital cardiopulmonary defect characterized by dextroposition of the heart, right lung hypoplasia, and anomalous right lung drainage into the systemic circulation. Scimitar syndrome is a congenital disorder often associated with other heart and lungs anomalies. Repair of Schnitar Syndrome Guerra NC(1), Pernot M(1), Nesseris G(1), Al-Yamani M(1), Roques X(1), Thambo JB(2), Kreitmann B(1), Roubertie F(3). We report a case of a 40-year-old woman with scimitar syndrome discovered on chest X . Scimitar syndrome (SS) can be repaired by different surgical techniques including direct implantation, intracardiac rerouting, and in situ pericardial channel to direct the Scimitar vein (SV) to the left atrium. Find out all about the causes, symptoms, treatment options and prognosis of the disease. This syndrome of partial or total anomalous pulmonary venous return of the right pulmonary veins to the inferior vena cava (IVC) was named 'Scimitar syndrome' by Neill in 1960 because the abnormal vessel can often be seen as a curvilinear density along the right heart border on plain chest radiography. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. : Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult 5. Combined minimally invasive repair of the adult form of ... Scimitar syndrome consists of a partial anomalous pulmonary venous drainage of the right lung, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply from the aorta or one of its branches to the right lung.1,2 There are both infantile and adult groups.2 Scimitar syndrome, like other congenital heart diseases, is commonly associated with atrial . Scimitar syndrome is as-sociated with other anomalies, including hypoplasia . It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. However, the surgical repair techniques for SS vary according to patients' anatomical and pathological features. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. A 15-year-old male recently diagnosed with scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel and patch augmentation of the inferior vena cava using PhotoFix® bovine pericardium. Scimitar syndrome is a complex constellation of vascular, bronchial, and parenchymal malformations of the right lung. From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Scimitar syndrome Provided by "Pediatric Heart Surgery — a reference for professionals" Pathophysiology. CONCLUSION: We conclude that age at detection of scimitar syndrome is important in predicting outcome. Scimitar syndrome; also known as congenital venolobar syndrome, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, and vena cava bronchovascular syndrome, is a rare congenital heart defect. Discussion. The shadow extends medially from the lateral superior position of the right lung and … Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. Scimitar syndrome is an infrequent congenital malformation. We applied a variant of the atrial switch technique using autologous pericardial … Considering the . The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. The authors present a novel technique for intracardiac baffle in scimitar syndrome that reroutes scimitar vein drainage to the left atrium by approximating t. The shadow extends medially from the lateral superior position of the right lung and … Restoration of the pulmonary valve reduces right ventricular volume overload after previous repair of tetralogy of Fallot. Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium). The authors postulate that their multipatch repair enlarges the left atrium; avoids acute angles within the . 1 The patient was referred to cardiothoracic surgery for surgical repair . Scimitar syndrome is a rare, congenital disease characterized by an anomalous (partial or entire) venous drainage of the right lung into the inferior vena cava (IVC), which results in the . Geggel and colleagues1 describe a multipatch repair technique for scimitar syndrome. Scimitar syndrome is a rare congenital cardiac defect characterized by anomalous pulmonary venous drainage from the right lung to the inferior vena cava . 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. 1 Introduction. Circulation. A review of our center's experience before March 2011 showed that one half of 36 patients who had a baffling or reimplantation procedure to repair scimitar syndrome developed pulmonary vein obstruction. Onalan MA et al. This tunneling technique allowed tension-free anastomosis and minimal Scimitar vein rotation. We present a newborn with diaphragmatic eventration, whose diagnosis of Scimitar syndrome was made after surgical repair. The mainstay of therapy for scimitar syndrome is surgical repair, as performed in the case of our patient, which improves outcome. Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. The association between scimitar syndrome and PH, at least in the preoperative setting, is well known [5-7]. The appearance on chest radiography resembles a curved Turkish sword, or scimitar. Scimitar syndrome is an infrequent congenital malformation. Author information: (1)Department of Cardiovascular Surgery, Bordeaux Heart University Hospital, University of Bordeaux II, Bordeaux, France 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 . 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. 1993 Nov; 88(5 Pt 2):II189-97. Vida and Guariento1 provide a timely, comprehensive review of pathophysiology, principles of diagnostic evaluation, and considerations for surgical repair for patients with scimitar syndrome. 2. Although most adult patients have no symptoms and a good prognosis, those with a large left-to-right shunt volume (pulmonary/systemic flow ratio > 1.5) or other cardiac diseases are usually symptomatic and are likely candidates for surgical treatment [].We present here a successful case of minimally invasive surgery via a . The presence of several anatomical variations such as remote infradiaphragmatic drainage of the SV and abnormal situs makes the repair more challenging with conventional repair techniques. described for repair of scimitar syndrome that in the short to intermediate term has not been associated with postoperative pulmonary vein obstruction. The Journal of Thoracic and Cardiovascular Surgery c Volume 152 . Successful surgical repair of the syndrome was first reported by Kirklin, Ellis, and Wood 3 in 1956. - Infantile Scimitar Syndrome Repair Braz J Cardiovasc Surg 2021;36(1):133-6 Fig. 2 The syndrome is almost invariably associated with other cardiac and extracardiac . Scimitar syndrome is a rare congenital disorder with an incidence of approximately 2 per 100 000 live births. 1 The scimitar vein normally enters the IVC below the level of the diaphragm, and the entire venous drainage of the right lung flows through the scimitar vein. Scimitar Syndrome. and RV failure require correction. These include occlusion of the systemic arterial collaterals, right pneumonectomy, and complete repair. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Scimitar Syndrome. See Editorial Commentary page e107. 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. We report a case of a 40-year-old woman with scimitar syndrome discovered on chest X . Complications of scimitar repair are stenosis, thrombosis and occlusion of the scimitar vein and its deviation. Central Message There is scant literature describing repair of an arrhythmia related to anomalous pulmonary venous return. References. 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 . 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 . Scimitar syndrome is a special type of partial anomalous pulmonary venous return (PAPVR). 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. Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava. Association between these conditions is rare. Conclusions Scimitar syndrome is a rare congenital malformation in adults, and although it has a better prognosis than the infantile forms, it continues to be a diagnostic challenge for cardiologists. Without surgery to correct this left-to-right shunt, many patients are at risk of . 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 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 . Scimitar syndrome is a rare form of anomalous pulmonary venous return with an estimated incidence of 2/100,000 in which all or part of the venous drainage from the right lung enters the inferior vena cava instead of the left atrium [].The syndrome derives its name from the classical appearance of the chest radiograph, in which the shape of the anomalous pulmonary vein resembles . Successful surgical repair of the syndrome was first reported by Kirklin, Ellis, and Wood 3 in 1956. Repair using an extracardiac conduit has rarely been performed. Up to 25% of cases are associated with other heart diseases. . Scimitar syndrome gets its name from the distinctive appearance they show when they are viewed on x-rays. Methods: From 1974 to 2012, 21 patients with scimitar syndrome underwent corrective surgery. On the x-rays they resemble a Middle Eastern weapon called the scimitar, which is a curved sword. The PAPVR in scimitar syndrome drains some, or all, of the right lung. There were no mortalities and no incidence of pulmonary vein obstruction following their multipatch repair technique. Postoperative pulmonary venous obstruction is prevalent, especially in the infants. 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. Several methods of repair have been described for routing the scimitar vein to the left atrium. As well, not only is there PAPVR, but . It has a varied presentation. 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 is a rare congenital disorder. Scimitar Syndrome DefinitionPage Contents1 Scimitar Syndrome Definition2 Scimitar Syndrome Synonyms3 Scimitar Syndrome Incidence4 Scimitar Syndrome Presentation5 Scimitar Syndrome Causes6 . Scimitar syndrome (SS) is an unusual condition of anomalous pulmonary venous return from the right lung to the inferior vena cava. The hallmark feature is anomalous venous drainage of the right lung that produces a distinctive image on anteroposterior chest roentgenogram (Fig 1 . Scimitar syndrome. 1 The hallmark of this syndrome is partial or complete anomalous venous return from the right lung, directly into the inferior vena cava (IVC), thereby creating a left-to-right shunt. 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. 4' 5 Dupuis and associates 6'7 identified two separate types of scimitar syndrome, the infantile and the adult 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.
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