Scimitar syndrome is a rare congenital heart disease which is characterized by an unusual arrangement of the pulmonary veins. There is a clear bimodal presentation of this syndrome. Scimitar syndrome scimitar syndrome is a rare pathologica subsel t of anomalou pulmonars drainagey wherei, oxn ygenated bloo frod m the righ lunt draing ints o the inferio venr a cava anatomica. In unrecognized cases the clinical symptoms usually manifest in the second to third decade of life with fatigue and dyspnea. Scimitar syndrome joseph junewick, md facr 03242012 history 4 year old male with dyspnea and asthmalike symptoms. Scimitar syndrome presenting in infancy the annals of. 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. The importance of localizing pulmonary veins in atrial septal defect closure. Scimitar syndrome lungs radiology reference article. This is most commonly caused by pulmonary hypertension due to cardiac andor right lung anomalies. The pathology of this disease was described in detail by park19 in 1912, but it was not until 1949 when. Scimitar syndrome and evolution of managements ncbi. Adult scimitar syndrome with severe pulmonary hypertension. The term derives from the curvilinear shadow created by the.
Scimitar syndrome the clinical manifestation and prognosis of scimitar syndrome depends on the amount of the resulting leftright shunt. During the course of her workup, transthoracic echocardiography demonstrated anomalous pulmonary venous return of the right upper and lower pulmonary veins to the inferior vena cava just above the diaphragm scimitar syndrome. Multimodality imaging of scimitar syndrome in adults. An unusual neonatal presentation of scimitar syndrome. 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 scimitar syndrome is a complex malformation of the heart, lungs, and blood vessels.
A rare case of scimitar syndrome with horseshoe lung. Echocardiographic view of the suprasternal notch in a patient with scimitar syndrome. Scimitar syndrome pulmonary venolobar syndrome is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. Treatment depends on symptom severity and may include correction of venous return in cases of severe heart failure. The clinical presentation may be insidious and associated with a number of cardiopulmonary anomalies. The anomalous vein is said to resemble a curved turkish sword scimitar. All structured data from the file and property namespaces is available under the. Scimitar syndrome in children and adults the annals of thoracic. Understanding sarahs scimitar syndrome authorstream. Surgery is the definitive treatment for scimitar syndrome. Repair of scimitar syndrome operative techniques in. This rare congenital malady was first described in autopsy specimens by cooper1 in.
Scimitar syndrome is a rare congenital anomaly consisting of partial anomalous pulmonary venous return from the right lung to the inferior vena cava ivc, hypoplasia of the right lung with. Scimitar syndrome is a rare congenital heart disease first reported in 1836 by cooper 3 and named by neill in 1960. Scimitar syndrome is a congenital deformity which consists of abnormal right side pulmonary venous drainage in the inferior vena cava, right lung hypoplasia, dextraposition of heart, and anomalous. Scimitar syndrome, also known as hypogenetic lung syndrome. Presentation1, radiological imaging of scimitar syndrome 1. Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. Brown scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava. Scimitar syndrome is a rarely seen congenital cardiopulmonary anomaly characterised by partial or complete anomalous pulmonary venous drainage into the inferior vena cava via an anomalous descending vein that runs parallel to the right border of the heart giving an appearance of a curved turkish sword or scimitar. Scimitar syndrome is a complex lesion in which the associated anomalies of the lung and heart are important determinants of management and prognosis.
Mild to moderate hypoplasia of the right lung results in displacement of the heart to the right side in a 6yearold girl with scimitar syndrome. With regard to the outcome of surgical approaches, the intracaval long baf. Get a printable copy pdf file of the complete article 2. There is an anomalous pulmonary vein on the right side because of its shape called scimitar. Scimitar syndrome is a rare entity within congenital heart disease. 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. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome. A case series of five infants with scimitar syndrome. Files are available under licenses specified on their description page. Scimitar syndrome belongs to the group of venolobar syndromes associated with pulmonary sequestration and congenital heart anomalies 1.
Presentations ppt, key, pdf logging in or signing up. Pdf scimitar syndrome is a constellation of rare, congenital, cardiopulmonary anomalies, comprising of partial anomalous pulmonary venous. Scimitar syndrome ss is a rare congenital heart defect chd with a frequency of cases per 100. We present a case of a 37 weeks gestation male infant with antenatal ultrasound findings of suspected partial anomalous pulmonary venous return papvr and coarctation of the aorta. 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. Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia. Correction of the scimitar syndrome, a rare cardiac venous. This anomalous pulmonary venous return can be either partial papvr or total tapvr. Repair of adult scimitar syndrome with an intraatrial conduit repair of adult scimitar syndrome with an intraatrial conduit. Scimitar syndrome international journal of pediatrics. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf. Scimitar syndrome is a congenital deformity which consists of abnormal right side pulmonary venous drainage in the inferior vena cava, right lung hypoplasia, dextraposition of heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung. Diagnosis scimitar syndrome discussion the lungs initially derive systemic. Scimitar syndrome is a rare complex congenital anomaly 1,000,000 live births, in which the anomalous pulmonary venous return of all or most of the right lung to the.
Clinical and echocardiographic characteristics of scimitar. Scimitar syndrome describes a specific subset of partial anomalous pulmonary venous return papvr in which an anomalous right. Scimitar syndrome is a complex constellation of vascular, bronchial, and parenchymal malformations of the right lung. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage scimitar syndrome. Incidence, treatment, and prognosis article pdf available in european journal of pediatrics 1672. We would like to show you a description here but the site wont allow us. Pulmonary hypertension in scimitar syndrome in infancy. The syndrome associated with papvr is more commonly known as scimitar. Presentation1, radiological imaging of scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left. Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung to the inferior vena. Scimitar syndrome is also called as the pulmonary venolobar, halaszs syndrome, mirrorimage lung syndrome, hypogenetic lung syndrome, epibronchial right pulmonary artery syndrome and venacava bronchovascular syndrome. Scimitar syndrome is a rare and complex congenital anomaly of the lung.
It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. Pdf scimitar syndrome or congenital pulmonary venolobar syndrome is a rare anomaly most commonly consisting of partial pulmonary venous drainage into. 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. The arrow points to the usual entry site of the pulmonary vein into the left atrium. The rare combination 35% of an association of papvr, hypoplasia of the right lung and dextroposition of the heart is designated scimitar syndrome. Scimitar syndrome is a rare congenital syndrome, occurring in per 100,000 live births, for which the sine qua non is anomalous venous drainage of the lung to the inferior vena cava fig. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on scimitar syndrome. 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. Scimitar syndrome is a relatively uncommon constellation of cardiopulmonary.