A case with pseudo-scimitar syndrome: "scimitar sign" with normal pulmonary venous drainage

The case reported here showed a radiological appearance of hypoplasia of the right lung, dextroposition of the heart, and a curved vascular shadow in the right lower lung field known as a scimitar sign. However, a computed tomography of the chest showed this abnormal vascular shadow draining into the left atrium (pseudo-scimitar sign). Therefore, in patients with a radiological appearance of the scimitar syndrome, computed tomography of the chest should be indicated to rule out the pseudo-scimitar sign.     

An unusual opacity of the right base

The scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable malformation of the right lung characterized by an abnormal right sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. We present a case in which this diagnosis was suspected on an abnormal routine chest radiograph in a 38-year-old asymptomatic woman. Most patients are asymptomatic; symptomatic patients have a marked left-to-right shunt or a severe congenital heart disease. They usually suffer from shortness of breath, asthenia or repeated chest infections. Usually, the posteroanterior chest radiograph can confirm the diagnostic. It shows the abnormal vein draining into the inferior vena cava as a curved vascular shadow with a scimitar like appearance. However, in some cases, when the scimitar vein is masked by the overlying cardiac shadow, computed tomography, angiography and magnetic resonance imaging can be helpful by showing the abnormal vein and its insertion into the inferior vena cava. Scimitar syndrome seldom necessitates surgical intervention. However, repeated lung infections can sometimes require lobectomy or pneumonectomy, left-to-right shunt vascular surgery to redirect the scimitar vein into the left atrium.     

Two Scimitar Veins in an Adult: Repair through a Right Thoracotomy without Cardiopulmonary Bypass

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. The appearance on chest radiography resembles a curved Turkish sword, or scimitar. Scimitar syndrome is associated with other anomalies, including hypoplasia of the right lung, dextroposition of the heart, anomalous systemic arterial supply to the right lung, and atrial septal defect. Clinical manifestations in infants include severe tachypnea and cyanosis. Adult patients may present with dyspnea and increased fatigue. Few adult cases have been reported in the medical literature. Classical surgical repair involves diversion of the scimitar venous flow into the left atrium with a baffle, requiring the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. Herein, we report the case of a 42-year-old woman with 2 scimitar veins who underwent corrective surgery at our center without the use of cardiopulmonary bypass. We also comment on the importance of a patient''s lung hypoplasia in the decision to repair the defect through a right thoracotomy.Key words: Cardiovascular surgical procedures/methods, dyspnea/etiology, heart atria/abnormalities/surgery, heart defects, congenital, pulmonary veins/abnormalities/surgery, patient selection, radiography, thoracic, scimitar syndrome/complications/diagnosis/physiopathology/surgery, treatment outcomeScimitar syndrome is defined as anomalous pulmonary venous drainage from the right lung to the inferior vena cava (IVC). The variability of this congenital condition has been well described.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. However, occasionally the scimitar vein drains only the middle or lower lobe. Anomalous drainage from the left lung has also been described.² The scimitar vein most often descends anterior to the hilum, grows larger as pulmonary veins drain into it, and curves slightly to the left to join the IVC. This creates a distinctive shape on chest radiography that resembles a curved Turkish sword, or scimitar, as described by Halasz and colleagues in 1956.³Classically divided into an infantile and a pediatric/adult form,^4,5 scimitar syndrome occurs in 2 of 100,000 births and is twice as common in females.⁶ Among affected persons, the scimitar sign is present in 70% of pediatric and adult patients but in less than 10% of infants. Coexisting anomalies can include hypoplasia of the right lung, systemic arterial blood supply to the right lung from the aorta (in 60% of cases), atrial septal defect (ASD, in 40% of affected patients), and dextroposition of the heart.⁶ Infants often present with severe tachypnea, cyanosis, and signs of congestive heart failure due to a substantial left-to-right shunt, resulting in failure to thrive. In contrast, children and adults are often asymptomatic or they may present with symptoms such as recurrent pneumonia, mild dyspnea, or increased fatigue. Dextroposition of the heart occurs less frequently in adults than in children but is more challenging to repair. Surgical techniques for repair of scimitar syndrome have varied widely, and few cases involving adults have been reported. Herein, we report the case of an adult patient with 2 scimitar veins who underwent corrective surgery through a right thoracotomy without cardiopulmonary bypass (CPB).     

Anomalous unilateral single pulmonary vein mimicking partial anomalous pulmonary venous return

We report a case in which the clinical findings of a partial anomalous pulmonary venous return were present but with termination of the anomalous pulmonary vein into the left atrium. There was hypoplasia of the right lung, dextroposition of the heart, and a loop-like vascular density in the right lung field. Cardiac catheterization revealed no shunt, and on pulmonary angiograms, a large common right pulmonary vein draining the entire right lung and entering the left atrium by way of a single orifice. Possible embryologic explanations include atresia of the right inferior pulmonary vein and persistence of the ipsilateral superior pulmonary vein.     

Atypical Mycobacterial Infection in Sequestrated Lung in an Infant Presenting with Chronic Pneumonitis and Recurrent Wheezing

An infant was referred to us for persistent cough and recurrent wheezing. Chest X‐ray showed an abnormal shadow in the right lower lobe. A volumetric contrast‐enhanced computerized tomography (CT) scan of the chest revealed an abnormal blood vessel extending from the descending aorta to the right lower lobe and anomalous venous drainage from the right lower lobe into inferior vena cava. Angiography confirmed the CT scan findings. Pulmonary sequestration associated with scimitar syndrome was subsequently diagnosed. Patient underwent right lower lobectomy and right inferior pulmonary vein was anastomosed into the left atrium. Pathological examination after surgery revealed epitheloid granuloma in the sequestrated lung parenchyma and myocobacteria in the draining lymph nodes. This is a rare case of pulmonary sequestration associated with scimitar syndrome and complicated by mycobacterial infection.     

Auffälliger Röntgenthorax bei jungem Feuerwehranwärter

An 18-year-old, asymptomatic male underwent a routine chest x-ray examination which showed a pathological result. It revealed a characteristic crescent-like shadow in the right lower lung field, resembling a muslim sword (scimitar), responsible for the name of the underlying disease. Scimitar syndrome is a rare congenital malformation with a wide clinical spectrum ranging from asymptomatic to severe disturbances manifesting in childhood. The typical findings are hypoplasia of the right lung with marked mediastinal shift to the right and dextrocardia, reduced right pulmonary artery perfusion with maintained bronchial connection, anomalous arterial supply to the right lower lobe from the aorta and abnormal draining of the enlarged right pulmonary vein into the pulmonary circulation, producing the characteristic radiographic “scimitar sign”. The development of right ventricular failure due to long-standing right ventricular overload and recurrent respiratory infections depend on the severity of anatomical abnormalities and functional disorders, sometimes requiring surgical intervention. Nowadays, sophisticated imaging techniques allow precise and rapid diagnosis of these complex abnormalities and their functional implications. The findings for our patient differed from the classic constellation, with a regular emptying of the enlarged right pulmonary vein into the left atrium, leading to a decreased burden on the central haemodynamics. In our very rare case of a scimitar syndrome variant, the functional disorders were negligible.     

A young fireman candidate with an abnormal chest x-ray

An 18-year-old, asymptomatic male underwent a routine chest x-ray examination which showed a pathological result. It revealed a characteristic crescent-like shadow in the right lower lung field, resembling a muslim sword (scimitar), responsible for the name of the underlying disease. Scimitar syndrome is a rare congenital malformation with a wide clinical spectrum ranging from asymptomatic to severe disturbances manifesting in childhood. The typical findings are hypoplasia of the right lung with marked mediastinal shift to the right and dextrocardia, reduced right pulmonary artery perfusion with maintained bronchial connection, anomalous arterial supply to the right lower lobe from the aorta and abnormal draining of the enlarged right pulmonary vein into the pulmonary circulation, producing the characteristic radiographic "scimitar sign". The development of right ventricular failure due to long-standing right ventricular overload and recurrent respiratory infections depend on the severity of anatomical abnormalities and functional disorders, sometimes requiring surgical intervention. Nowadays, sophisticated imaging techniques allow precise and rapid diagnosis of these complex abnormalities and their functional implications. The findings for our patient differed from the classic constellation, with a regular emptying of the enlarged right pulmonary vein into the left atrium, leading to a decreased burden on the central haemodynamics. In our very rare case of a scimitar syndrome variant, the functional disorders were negligible.     

The relationship between scimitar syndrome, so-called scimitar variant, meandering right pulmonary vein, horseshoe lung and pulmonary arterial sling

We report a case in which a meandering right pulmonary vein connecting to the left atrium is associated with hypoplasia of the right lung, horseshoe lung, abnormal pulmonary lobation, and abnormal branching of the pulmonary arteries. We discuss its relationship to the so-called scimitar variant, and to the scimitar syndrome itself.     

Hypoplastic Right Lung Associated with Right Pulmonary Vein Stenosis and Systemic Collateral

We present a case of a 6-month-old girl diagnosed as unilateral pulmonary vein stenosis (right inferior pulmonary vein) with dextroposition of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, collateral from the descending aorta supplied right lower lung, connection between collateral and right pulmonary artery with retrograde flow from right pulmonary artery into main pulmonary artery, and right superior and middle pulmonary vein agenesis. A combined modality of imaging including chest film, echocardiography, multidector computerized tomography, and the cardiac catheterization nicely demonstrated all of the unique and rare congenital abnormalities.     

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as “the scimitar sign”, is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.     

Horseshoe lung associated with scimitar syndrome and pleural lipoma

Horseshoe lung is a rare congenital anomaly characterized by the presence of unilateral pulmonary hypoplasia and a midline isthmus bridging the right and the left lung. Almost all cases of horseshoe lung are associated with the scimitar syndrome. The scimitar syndrome, sometimes called the congenital pulmonary venolobar syndrome, is a rare but well-described constellation of cardio-pulmonary anomalies. The characteristic abnormality is anomalous pulmonary venous return from a part of or the entire right lung to the inferior vena cava. We present a case of horseshoe lung associated with scimitar syndrome and pleural lipoma in an adult admitted for acute chest pain and chronic dyspnea.     

A case of PAGOD syndrome with hypoplastic left heart syndrome

PAGOD syndrome is an extremely rare congenital malformation complex involving multiple organs. These include pulmonary artery and lung hypoplasia, diaphragm defects, omphalocele, sex reversal or ambiguous genitalia, and complex cardiac defects. Eight cases have been reported to date. We report a case of PAGOD syndrome that is manifested by right diaphragm eventration, horseshoe lung with right lung hypoplasia, hypoplastic left heart syndrome (mitral atresia, aortic atresia), scimitar syndrome, agonadism with ambiguous genitalia. A karyotype revealed normal 46-XY. This patient received a modified Norwood procedure for hypoplastic left heart syndrome as an initial palliation and bidirectional cavopulmonary anastomosis as a second stage of operation. The postoperative courses were uneventful. This patient is waiting for Fontan operation.     

Scimitar syndrome

We studied 15 cases of the scimitar syndrome. The diagnosis was suspected in 87% of the patients by x-rays studies. Bronchography is useful in order to detect bronchial anomalies, such as hypoplasia or agenesis of the medial lobe which is responsible for the left isomerism. The hemodynamic study is important in symptomatic patients in order to demonstrate associated congenital heart disease as well as the degree of pulmonary hypertension. Angiography is fundamental in determining the anomalous venous drainage of the right lung, whether complete or partial, and in demonstrating the exact site of the connection which may be to the right atrium or more frequently to the inferior vena cava above or below the diaphragm. There are variant forms in so far as the pathway, extension and connection of the anomalous venous trunk which gives rise to the scimitar sign. This syndrome is due to a persistent connection of the right pulmonary veins to the proximal portion of the right vitelin vein which normally forms the suprahepatic segment of the inferior vena cava. Asymptomatic patients should have medical treatment symptomatic ones with an associated congenital heart disease and moderate pulmonary hypertension must be subject to surgery.     

Pulmonary Sparganosis: Tunnel Sign and Migrating Sign on Computed Tomography

A 77-year-old woman presented at our hospital to undergo a close examination of an abnormal shadow which was observed on a chest radiograph. Contrast-enhanced computed tomography (CT) images in the lung window revealed a tortuous tunnel structure (tunnel sign), which was suspected to be the migration path of a parasite. Furthermore, CT images in the mediastinal window showed a linear filling defect from the right inferior pulmonary vein to the venous ostium in the left atrium (migrating sign), which was suspected to be a migrating parasite in the pulmonary vein. Tunnel and migrating signs on chest CT images were helpful in diagnosing pulmonary sparganosis.     

A case of right pulmonary hypoplasia with congenital diaphragmatic hernia and dextrocardia]

Chest X-ray of a 28-year-old woman revealed an abnormal shadow in the right lower lung field and dextrocardia, for which detailed investigation was performed. Since the CT number of the tumor shadow corresponded to that of the liver on chest CT, diaphragmatic hernia of the liver was suspected, and was confirmed by MRI and angiography of the abdomen. In addition, the pulmonary artery and vein were hypoplastic, and angiography of the pulmonary artery demonstrated pulmonary hypoplasia. This case was considered to have primary pulmonary hypoplasia, because the dextrocardia was considered to have occurred secondary to pulmonary hypoplasia and the diaphragmatic hernia of the liver was not sufficiently large to cause pulmonary hypoplasia. Pulmonary hypoplasia first diagnosed in adulthood is rare, with a clinical course and roentgenographic appearance differing from those of pulmonary hypoplasia in children.     

A "late" scimitar syndrome. Diagnostic contribution of cardiac computed tomography

A 51-year-old female hospitalized with a non-specific colitis, presented a crescent-like shadow in the right lower lung accompanied by a reduced right lung volume on a routine chest x-ray. There was no family history of congenital heart disease. The initially performed, noninvasive, contrast enhanced cardiac CT (electron-beam tomography [EBT]) proved the suspected diagnosis of a partial, anomalous pulmonary, transdiaphragmatic vein drainage (APVD) in combination with a hypoplastic right lower lobe and dextrocardia. These findings are in accordance with scimitar syndrome. Regarding to the clinical situation with symptoms like slowly progressive dyspnea on exertion and low exercise tolerance for the last 2 years and an invasively documented left-to-right shunt ratio >50% (Qp:Qs = 2.6 : 1) surgical repair was recommended. The anomalous vein was connected to the left atrium creating a "neo-septum". On a postoperative checkup after 9 months the patient is without any medication, symptoms during moderate activity are relieved, exercise tolerance was substantially better and noninvasive imaging visualized the corrected drainage of the anomalous vein to the left atrium.     

Right-sided scimitar syndrome in a patient with a single aortic trunk and coronary-cameral venous fistula

Scimitar syndrome (SS) is a rare congenital anomaly characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persistent left superior vena cava, and dextroposition of the heart. We report a rare variant of SS in a 44-year-old man together with a single aortic trunk, as well as a coronary-cameral venous fistula.     

Asymmetric pulmonary perfusion causing unilateral pulmonary edema as a complication of acute myocardial infarction

Acute unilateral pulmonary edema is an unusual clinical condition occasionally associated with severe mitral valve insufficiency. We describe a patient diagnosed as having unilateral pulmonary edema after an acute anterior myocardial infarction. Echocardiograms performed in the acute phase ruled out mitral insufficiency. A perfusion lung scan showed left-sided pulmonary hypoperfusion. The diagnosis of acquired hypoplasia of the left pulmonary artery tree was made by chest computed tomography. The pathogenesis and differential diagnosis are discussed.     

Right lung cancer with partial anomalous pulmonary venous return and absent right upper lobe

When a vascular shunt is present, major lung resection may cause acute right heart failure. We report a case of right lung cancer with ipsilateral partial anomalous pulmonary venous return (PAPVR) and absent right upper lobe managed by pneumonectomy. A 48-year-old former smoker was diagnosed as right lung cancer; chest computed tomography (CT) revealed an anomalous right pulmonary vein draining into inferior vena cava. Bronchus of right upper lobe was not found in fiberoptic bronchoscopy. Right pneumonectomy was performed. Pathological examination revealed a T2aN2M0 squamous lung cancer. The patient tolerated well without notable descending of arterial oxygen pressure.     

Single left pulmonary vein with normal pulmonary venous drainage: Association with partial anomalous pulmonary venous return

An unusual case of a single left pulmonary vein draining the left lung and joining the left atrium without venous obstruction is reported. This anomaly occurred in association with partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava. A similar case has been previously reported from our laboratory. The diagnosis of a single left pulmonary vein can be made by routine chest roentgenography because of the charácteristic radiologic appearance of this anomaly. It is important to distinguish this benign entity from more ominous pulmonary radiodensities.