The patient did not have any other associated congenital heart defects chd incomplete scimitar syndrome. The rightsided chambers of the heart and pulmonary arteries were dilated because of lefttoright shunting. Scimitar syndrome is a rare disorder of the pulmonary circulation in which the pulmonary vein of the right lung drains into the inferior vena cava or the right atrium, instead of the left atrium, and is accompanied by other cardiopulmonary anomalies. To the editor scimitar syndrome, also known as pulmonary venolobar syndrome, is a rare congenital heart condition that encompasses an array of anatomical abnormalities, including. The delay in diagnosis can be resolved by an early chest radiograph. 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. This accounts for postnatal descriptions of a sequestrated lung in scimitar. Repair of scimitar syndrome using an extracardiac conduit.
Associated pulmonary and vascular anomalies have been reported in cases of scimitar syndrome. Presentation1, radiological imaging of scimitar syndrome. Scimitar syndrome is a rare but well defined disorder, involving anomalies of lung and pulmonary vein1. Correction of the scimitar syndrome, a rare cardiac venous. Scimitar syndrome is characterized by partial or total anomalous. Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons. Scimitar syndrome was suspected in 5 cases because of dextroversion, and diagnosed by color doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava. Scimitar syndrome ss is a rare congenital anomaly characterized by a partial usually the right lower lobe or complete unilateral anomalous pulmonary venous drainage in association with ipsilateral lung hypoplasia. This group is for people who have scimitar syndrome, had scimitar. Scimitar syndrome is a rare congenital anomaly characterised by abnormal venous drainage of the lung to the inferior vena cava via a scimitar vein. A chest radiography of a patient with scimitar syndrome showing a leftside anomalous pulmonary vein panel a, which was confirmed by magnetic resonance imaging panel b.
An unusual neonatal presentation of scimitar syndrome. Subsequently, two systems of classification evolved to describe the malformed heart. 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 on plain chest radiography. The radiological description scimitar sign may occur in isolation which is more frequently encountered. Incidental partial anomalous pulmonary venous connection in left lung cancer. Three of the cases had congenital cardiovascular abnormalities not usually regarded as part of the scimitar syndrome, namely, ventricular septal defect, abnormalities of the aortic arch, and abnormal relationship of the pulmonary arteries and bronchi.
Scimitar syndrome ss encompasses a constellation of malformations with as a most. Clinical and echocardiographic characteristics of scimitar. We present a seven year old girl with a systolic murmur who was diagnosed as having a scimitar syndrome with unusual drainage of the right pulmonary veins. Scimitar syndrome is a rare congenital cardiovascular anomaly with an incidence of per 1,00,000 live births. The incidence of scimitar syndrome is estimated at 1 to 3100,000 live births. There is a clear bimodal presentation of this syndrome with either an infantile manifestation or a pediatricadult form. In patients with scimitar syndrome, right pulmonary artery hypoplasia is considered to lead to right lung hypoplasia because of decrease in blood flow.
The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava ivc creating a tubular opacity paralleling the right cardiac border on chest radiography which resembles a curved turkish sword or scimitar. This anomalous pulmonary venous return can be either partial papvr or total. The syndrome associated with papvr is more commonly known as scimitar. A 70yearold woman presented with worsening shortness of breath.
Late presentation and successful treatment of classical scimitar. The pathology of this disease was described in detail by park 19 in 1912, but it was not until 1949 when dotter et al. Harre and forrest for their comment on our article. Scimitar syndrome is a complex constellation of vascular, bronchial, and parenchymal malformations of the right lung.
In unrecognized cases the clinical symptoms usually manifest in the second to third decade of life with fatigue and dyspnea. The curved course of the anomalous right pulmonary vein gives the characteristic appearance of a turkish sword scimitar on chest xray. Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia. The presence of several anatomical variations such as remote infradiaphragmatic drainage of the sv and abnormal situs makes the repair more challenging. Scimitar syndrome is a rare congenital cardiac anomaly. Scimitar, or pulmonary venolobar, syndrome is a rare but wellknown congenital cardiovascular defect that includes a hypoplastic right pulmonary artery and right lung, which leads to displacement of cardiac structures into the right hemithorax, anomalous systemic arterial supply to the right lung, and a characteristically curved anomalous right pulmonary vein that drains. Combined minimally invasive repair of the adult form of. Papvr occurs when some of the pulmonary veins, the veins that drain the lungs, return to the right side of the heart instead of to the left atrium. Scimitar syndrome definitionpage contents1 scimitar syndrome definition2 scimitar syndrome synonyms3 scimitar syndrome incidence4 scimitar syndrome presentation5 scimitar syndrome causes6. After four days of treatment, the antibiotics were changed to.
Scimitar syndrome was first described by chassinat in 1836. An unusual presentation of scimitar syndrome in a military. Four neonates with scimitar syndrome are presented. Partial anomalous pulmonary venous return papvr is a rare congenital malformation. Scimitar syndrome is a rare vascular anomaly of both pulmonary hypoplasia and partial anomalous pulmonary venous return. Scimitar syndrome is a rare variant of anomalous pulmonary venous return that is usually diagnosed in infanc, but may present in adults with symptoms of dyspnea on exertion, syncope, or heart failure. Pulmonary artery hypertension and hemoptysis both are uncommon features of. Scimitar syndrome is a rare congenital heart defect where blood from the right lung is returned to the wrong side of the heart. 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. Scimitar syndrome a variant of partially anomalous pulmonary venous return or papvr is a rare congenital heart defect is characterized by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. 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. Adult scimitar syndrome with severe pulmonary hypertension.
Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. Repair of scimitar syndrome operative techniques in. Dear rach, scimitar syndrome is a special type of partial anomalous pulmonary venous return papvr. 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 characterized an anomalous right pulmonary venous return and cardiovascular compromise 3. An unusual neonatal presentation of scimitar syndrome ncbi.
Children may present with tachypnea early in life or with repeated chest infection at any age. The term scimitar refers to the fact that the vertically oriented tubular structure resembles the turkish sword known as a scimitar. Such findings typify the scimitar or pulmonary venolobar syndrome 1. Its presentation in childhood requires early surgical interventions to correct the. On a chest radiograph, the abnormal vessel configuration produces a scimitar shaped shadow.
The sequential segmental variant of the system, emanating from the socalled european school led by anderson and colleagues in the 1970s,4, 5 combines this morphologic information with the ways in which the segments are joined, or not joined, to each other, and then to their relationships. Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made. However, there are no reports wherein the change was actually detected. A ct angiogram shows a scimitar shaped vein draining the entire right lung to the inferior vena cava at its junction with the right atrium. Scimitar syndrome and pulmonary sequestration ps have overlapping features. The infracardiac variant with the right lobe of the lung draining to the inferior vena cava ivc is called scimitar syndrome.
May 06, 2020 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 cava, right. Scimitar syndrome congenital pulmonary venolobar syndrome. Once a ct confirms the diagnosis, patients should be referred. It is important to diagnose or at least to rule the syndrome as management depends upon the severity of different components. Scimitar syndrome the clinical manifestation and prognosis of scimitar syndrome depends on the amount of the resulting leftright shunt.
The anomalous vein is said to resemble a curved turkish sword scimitar. This lefttoright shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of. Adult patients are often asymptomatic, while infants present with significant symptoms. The right inferior pulmonary vein is joining the posterolateral aspect of the. In adult life, it usually presents either as recurrent chest infection andor exertional dyspnea. With regard to the outcome of surgical approaches, the intracaval long baf. The objectives of this study were to determine the anatomic and physiologic factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome. The anomalous vein is referred to as the scimitar, because it resembles a turkish sword on chest radiography figure 1. Novel technique of surgical management of scimitar syndrome. This lefttoright shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of recurrent pneumonia or significant. The anomalous vein is referred to as the scimitar, because it resembles a turkish sword on.
Thus, the exact developmental mechanism of right pulmonary artery hypoplasia and right lung hypoplasia in patients with scimitar syndrome is unclear. Pdf scimitar syndrome is a constellation of rare, congenital, cardiopulmonary. Although most adult patients have no symptoms and a good prognosis, those with a large lefttoright shunt volume pulmonarysystemic flow ratio 1. Scimitar syndrome an overview sciencedirect topics. Scimitar syndrome is a rare congenital cardiovascular anomaly in which a part or the entire right lung is drained by pulmonary veins connecting to the vena cava, in combination with hypoplasia of the right lung, pulmonary hypertension and potential other cardiac defects 14. It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return. The most constant feature is considered the anomalous venous drainage of the right lung into the inferior vena cava. The scimitar syndrome is a complex malformation of the heart, lungs, and blood vessels. Unusual course of scimitar syndrome preceded by lung. Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava.
The infantile subtype presents before one year of age and the adult variant is also usually diagnosed in childhood. How to pronounce scimitar in english cambridge dictionary. The unique technique of surgery in this patient was appropriate to the unusual. This rare congenital malady was first described in autopsy specimens by cooper1 in london and chassinat2 in paris in 1836. The shadow extends medially from the lateral superior position of the right lung and. There is a clear bimodal presentation of this syndrome with either an. Since then clinical observations have been published with increasing frequency, 127 indicating that the scimitar syndrome. The name scimitar comes from the anomalous pulmonary vein that courses along the right cardiac margin as a curvilinear shadow and is said to resemble a scimitar, or turkish sword. Scimitar syndrome and htype tracheoesophageal fistula in. They are often an incidental finding, however, surgical repair may be needed to avoid comp. Additional characteristics of this syndrome such as hypoplasia and abnormalities of the vascular supply to the right lung, dextrocardia and abnormalities of the bronchial segmentation are common. Scimitar syndrome is a rare congenital abnormality resulting from rightsided. Unique technique of surgery in an unusual variety of scimitar. Jan 21, 2017 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, also known as congenital pulmonary venolobar syndrome, is. The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. Imperforate anus associated with anomalous pulmonary venous. The scimitar syndrome is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. Scimitar syndrome is an anomaly of pulmonary venous drainage into the venous circulation, resulting in a lefttoright shunt. Scimitar, venolobar, or hypogenetic lung syndrome is a rare congenital anomaly of pulmonary venous return,1 in which the right pulmonary vein drains anomalously into the ivc. 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. Its name refers to the tubular opacity typically paralleling the right cardiac border that can be. Atypical infantile form of scimitar syndrome with bronchomalacia atypical infantile form of scimitar syndrome with bronchomalacia. Listen to the audio pronunciation in the cambridge english dictionary. Successful surgical repair of the syndrome was first reported by kirklin, ellis, and wood 3 in 1956.
Scimitar syndrome definition of scimitar syndrome by. Right partial anomalous pulmonary venous connection found during lobectomy for coexisting lung cancer and tuberculosis. Group i comprises the adult form, which shows no pulmonary artery hypertension pah and involves a small septal defect. It is a type of partial anomalous pulmonary venous return and is one of several findings in congenital pulmonary venolobar syndrome. Radiographic features of the scimitar syndrome radiology. Patients in whom the diagnosis was made during the first year of life infantile group, n 19 had more severe symptoms and had a higher incidence of heart failure 1119. Repair of adult scimitar syndrome with an intraatrial conduit repair of adult scimitar syndrome with an intraatrial conduit. On the chest radiograph, the vein produces a vascular shadow to the right of the heart that descends toward the diaphragm, resembling a scimitar, which is a short curved turkish sword. Dec 21, 2015 the vertical vein is similar to a scimitar 2, a turkish sword. Feb 22, 2017 findings are consistent with scimitar syndrome.
May 06, 2020 scimitar syndrome can have variable clinical manifestations. We would like to show you a description here but the site wont allow us. Mar 25, 2010 scimitar syndrome is a rare congenital anomaly characterized by total or partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava. In general, infants presenting in heart failure have a greater number of associated anomalies and their prognosis is much worse. 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. Prenatal findings and differential diagnosis of scimitar. Louis childrens hospital who presented with symptoms attributable to scimitar syndrome. Scimitar syndrome is a form of partial anomalous pulmonary venous drainage. The hypoplastic right lung and anomalous pulmonary vein confirmed that she had congenital pulmonary venolobar syndrome also known as scimitar syndrome. Scimitar syndrome belongs to the group of venolobar syndromes associated with pulmonary sequestration and congenital heart anomalies 1. The syndrome associated with papvr is more commonly known as scimitar syndrome. The papvr in scimitar syndrome drains some, or all, of the right lung. An elevated right hemidiaphragm, cardiac dextroposition, or presence of scimitar sign should alert clinician to suspect scimitar syndrome.
Late presentation of scimitar syndrome mayo clinic. Thirtytwo patients with scimitar syndrome were seen in the period between 1975 and 1995. Scimitar syndrome was first described in 1836 by george cooper during the autopsy of a 10monthold infant. It is not intended to be and should not be interpreted as medical advice or a diagnosis of any health or fitness problem, condition or disease. The scimitar syndrome is a congenital anomalous connection of the pulmonary vein with the inferior vena cava. Find out all about the causes, symptoms, treatment options and prognosis of the disease. Patients in whom the diagnosis was made during the first year of life infantile group, n 19 had more severe symptoms and had a higher incidence. Scimitar syndrome forms only 3% to 5% of all cases of partial anomalous pulmonary venous drainage. Scimitar syndrome or bronchopulmonary sequestration.
The hallmark feature is anomalous venous drainage of the right lung that produces a distinctive image on. Scimitar syndrome has a variable presentation based on the age at which the diagnosis is made. 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. Dec 06, 2012 scimitar syndrome is a condition that can occur as an innate disorder or may develop at a later age of an individual. 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. Scimitar syndrome is a rare finding among malaysian population. The diagnosis is made through radiographic findings, and. The scimitar syndrome is a rare and variable congenital malformation. Scimitar syndrome presenting in infancy the annals of. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. Scimitar syndrome and anesthetic implications sciencedirect. A rare case of scimitar syndrome with horseshoe lung.
In this twentytwoyearold soldier with prior combat deployments, ccta was vital in the diagnosis of scimitar syndrome. Scimitar syndrome is a rare form of partial anomalous pulmonary venous. 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 cava, right. 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. Scimitar syndrome university of medicine and dentistry of. 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. In view of the fact that only 54 cases have been reported in the world literature 1 and 7 patients with this malformation have been studied at the university of minnesota. A case series of five infants with scimitar syndrome.
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 variant of papvd, partial anomalous pulmonary venous connection or partial anomalous pulmonary venous return, the syndrome has a prevalence of 0. Scimitar syndrome is a rare but well described constellation of cardiopulmonary anomalies. There is still debate about which technique to use in treating scimitar syndrome. Scimitar syndrome on chest x ray postgraduate medical journal. Pdf scimitar syndrome refers to a form of anomalous pulmonary venous drainage which implies partial or total failure of the pulmonary veins. This anomalous pulmonary venous return can be either partial papvr or total tapvr.
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