Scimitar syndrome definition, symptoms, causes, treatment. Mearadji international foundation for pediatric imaging aid rotterdam, the netherlands. Computed tomography ct helped in demonstrating the hyparterial relationship of the right bronchus to the pulmonary artery, the course and termination of the scimitar vein, the mediastinal shift, unusual fissures, and abnormal lobation and bronchial distribution. It is characterized an anomalous right pulmonary venous return and cardiovascular compromise 3. 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. Adult scimitar syndrome with severe pulmonary hypertension. Adult patients are often asymptomatic, while infants present with significant symptoms. 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.
The existence of these symptoms suggested the scimitar syndrome in the patient, and the patient was operated on june, 2014. 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. Treatment depends on symptom severity and may include correction of venous return in cases of severe heart failure. Pulmonary artery hypertension and hemoptysis both are uncommon features of. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome. Free fulltext pdf articles from hundreds of disciplines, all in one place scimitar syndrome in an adult. The infantile form is particularly complex and repair of the anomalous pulmonary vein has not been successful. Scimitar syndrome, anomalous pulmonary venous drainage. They are often an incidental finding, however, surgical repair may be needed to avoid comp. Radiographic features of the scimitar syndrome radiology. Scimitar syndrome as a differential diagnosis in a child. Dec 17, 2009 background scimitar syndrome is a rare association of congenital cardiopulmonary anomalies, and first described by cooper in 1836 1,2. Alternatively as in our patient, the syndrome may remain clinically silent and only be discovered incidentally in adulthood. Case report, case study by journal of evolution of medical and dental sciences.
It is a variant of a partial anomalous pulmonary venous return that results in a lefttoright shunt, with a characteristic anatomic feature that resembles a backsword or a saber with a. The infracardiac variant with the right lobe of the lung draining to the inferior vena cava ivc is called scimitar syndrome. The complete reference for scimitar syndrome 1st edition. Anatomy, epidemiology, diagnosis and treatment gives the complete picture of this rare syndrome that is usually treated with tactics such as imaging.
Scimitar syndrome ss is a rare congenital heart defect chd characterized by anomalous venous drainage of part or all of the right lung to the inferior vena cava, variable right lung hypoplasia, and variable systemic blood supply to part of the right lung. Scimitar syndrome ss is a rare congenital heart defect chd with a frequency of cases per 100. Scimitar syndrome definitionpage contents1 scimitar syndrome definition2 scimitar syndrome synonyms3 scimitar syndrome incidence4 scimitar syndrome presentation5 scimitar syndrome causes6. The complete reference for scimitar syndrome by vladimiro. The pathology of this disease was described in detail by park19 in 1912, but it was not until 1949 when dotter et al. Scimitar syndrome belongs to the group of venolobar syndromes associated with pulmonary sequestration and congenital heart anomalies 1. Scimitar, or pulmonary venolobar, syndrome is a rare but wellknown. The scimitar sign is a characteristic chest roentgenographic finding of a crescentlike shadow in the right lower lung field that is produced by the anomalous venous channel. 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. In unrecognized cases the clinical symptoms usually manifest in the second to third decade of life with fatigue and dyspnea. Scimitar syndrome is a rare congenital heart defect. Aug 17, 2017 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. The scimitar syndrome is a rare, congenital disorder which is seen more often in girls than boys.
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. A 70yearold woman presented with worsening shortness of breath. Apr 11, 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. Horseshoe lung is one of the rare congenital anomalies of the lung. On the xrays they resemble a middle eastern weapon called the scimitar, which is a curved sword. Natural history and clinical outcome of uncorrected scimitar. The rare combination 35% of an association of papvr, hypoplasia of the right lung and dextroposition of the heart is designated scimitar syndrome. A band of pulmonary parenchyma is formed extending between the right and left lungs. Scimitar syndrome an overview sciencedirect topics. In this coronal oblique view, superior and inferior pulmonary venous radicals are seen draining to anomalous or scimitar vein and into ivc i at diaphragmatic level. The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. The infantile subtype presents before one year of age and the adult variant is also usually diagnosed in childhood. Scimitar syndrome and anesthetic implications sciencedirect. Scimitar syndrome lungs radiology reference article.
The pulmonary tissue can be seen either anterior to the aorta or posterior to the pericardium at the caudal end pathology. 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. Scimitar syndrome congenital pulmonary venolobar syndrome. The defining abnormality of this condition is a partial anomalous pulmonary venous drainage of a part of or the entire right lung into the inferior vena cavaatrial junction, the inferior vena cava, or the right atrium. Pdf scimitar syndrome or congenital pulmonary venolobar syndrome is a rare anomaly most. 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. Echocardiographic view of the suprasternal notch in a patient with scimitar syndrome.
The diagnosis is made through radiographic findings, and. Presentation1, radiological imaging of scimitar syndrome 1. Scimitar syndrome and evolution of managements ncbi. Scimitar syndrome, a rare anomaly of partial pulmonary venous drainage into the hepatic portion of the inferior vena cava, detected in a tendayold neonate who presented clinically with respiratory distress and diagnosed on ultrasound of the thorax is being reported here. Scimitar syndrome causes scimitar syndrome is a congenital disease which occurs during fetal development within womb of mother. Patients may develop symptoms in infancy from the shunt or associated cardiac or respiratory developmental abnormalities. 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. 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. Computed tomography ct helped in demonstrating the hyparterial relationship of the right bro. Partial anomalous pulmonary venous return papvr is a rare congenital malformation.
Scimitar syndrome definition of scimitar syndrome by. Jan 21, 2017 presentation1, radiological imaging of scimitar syndrome 1. Scimitar syndrome can be associated with congenital cardiovascular defects. 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.
In the infantile form, anomalous pulmonary venous return is. This lefttoright shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of recurrent pneumonia or significant. 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. To analyze the clinical status of patients with uncorrected scimitar syndrome in a. Correction of the scimitar syndrome, a rare cardiac venous. The anomalous vein is said to resemble a curved turkish sword scimitar. Lung sequestration and scimitar syndrome imaging approaches m. Scimitar syndrome gets its name from the distinctive appearance they show when they are viewed on xrays. Scimitar syndrome is a rare vascular anomaly whereby a partial anomalous pulmonary venous drainage to the inferior vena cava results in lefttoright shunt. 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.
Data were acquired during free breathing with respiratory gating. Four new cases of scimitar syndrome illustrate the complex derangement of the right lung architecture. The shadow extends medially from the lateral superior position of the right lung and. A rare case of scimitar syndrome with horseshoe lung. However, surgery is not free of complications and, based on the suboptimal. Scimitar syndrome is a rare but well defined disorder, involving anomalies of lung and pulmonary vein1.
Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava. Group i comprises the adult form, which shows no pulmonary artery hypertension pah and involves a small septal defect. Pulmonary sequestration pulmonary sequestration ps is defined as a segment of lung parenchyma separated from the bronchial tree. Scimitar syndrome repair in adults the annals of thoracic surgery. Reformatted oblique section through 3d data set using gadoliniumdtpa contrast enhancement.
Scimitar syndrome presenting in infancy the annals of. Learn about its variable presentation and the treatment options. The pathology of this disease was described in detail by park19 in. This syndrome is a form of anomalous pulmonary venous drainage apvd. The term scimitar refers to the fact that the vertically oriented tubular. Scimitar syndrome, also known as pulmonary venolobar syndrome, is a rare congenital heart condition that encompasses an array of anatomical abnormalities, including. The syndrome associated with papvr is more commonly known as scimitar. A case series of five infants with scimitar syndrome. This anomalous pulmonary venous return can be either partial papv. Scimitar syndrome is a rare vascular anomaly of both pulmonary hypoplasia and partial anomalous pulmonary venous return. Treatment is surgical and outcome is dependent on the nature and severity of. Scimitar syndrome is a rare association of cardiopulmonary anomalies.
It is important to diagnose or at least to rule the syndrome as management depends upon the severity of different components. Very few cases in adults are reported in the literature. The arrow points to the usual entry site of the pulmonary vein into the left atrium. There is a clear bimodal presentation of this syndrome with either an.
The book goes beyond imaging, providing a pathological and clinical description of this rare syndrome. The name scimitar comes from the anomalous pulmonary vein that courses along the. Treatment may be medical, surgical or combined in majority of cases. Health, general congenital heart defects care and treatment case studies diagnosis congenital heart. 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.
It is also referred to as partial pulmonary venolobar syndrome. It has a wide spectrum of presentation and may sometimes only present in adulthood with symptoms of wheeze, recurrent chest infections, or pulmonary hypertension. 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. Scimitar syndrome is a rare congenital heart disease first reported in 1836 by cooper 3 and named by neill in 1960. Aug 12, 2014 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. Find out all about the causes, symptoms, treatment options and prognosis of the disease. Scimitar syndrome symptoms, prognosis, treatment, life. In the cardiac operation, the anastomosis of the abnormal pulmonary veins in the right side with the left atrium and the closure of asd were carried out. Presentation1, radiological imaging of scimitar syndrome. Scimitar syndrome is a rare congenital cardiac anomaly. It results in a partial to total anomalous venous drainage of the right lung into the inferior vena.
From department of radiology, university of brescia, italy. The hypoplastic right lung and anomalous pulmonary vein confirmed that she had congenital pulmonary venolobar syndrome also known as scimitar syndrome. The scimitar syndrome is a congenital anomalous connection of the pulmonary vein with the inferior vena cava. Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. The scimitar syndrome is a complex malformation of the heart, lungs, and blood vessels.
Clinical and echocardiographic characteristics of scimitar. Scimitar syndrome, also known as hypogenetic lung syndrome,is characterized by a hypoplastic lung that is drained. Puigmassana and revuelta 89 described use of free wall of the right. H treatment of anomalous pulmonary venous connections in association with interatrial communications, surgery 39. 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 a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection. Children may present with tachypnea early in life or with repeated chest infection at any age. In adult life, it usually presents either as recurrent chest infection andor exertional dyspnea. Scimitar syndrome the clinical manifestation and prognosis of scimitar syndrome depends on the amount of the resulting leftright shunt. With regard to the outcome of surgical approaches, the intracaval long baf. So it is concluded that occurrence of scimitar syndrome is totally related to family history. 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 dextroposition of the heart, and anomalous systemic arterial supply from the abdominal aorta to a portion of the right lung usually lower lobe.
Scimitar syndrome with unilobar right lunga case report. 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. Abstract scimitar syndrome is a rare and complex congenital anomaly, which is characterized by the image of a turkish sword on the chest xray. There is an anomalous pulmonary vein on the right side because of its shape called scimitar vein see text, white arrows draining into the inferior vena cava. 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 inferior vena cava just below or above the right hemidiaphragm creates the image of a turkish sword on the chest xradiography. The exact cause behind this syndrome is still unknown.
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