Scimitar syndrome: MDCT imaging revisited

Scimitar syndrome refers to a form of anomalous pulmonary venous drainage which implies partial or total failure of the pulmonary veins to reach the left atrium.AimTo emphasize the role of MDCT in the diagnosis of Scimitar syndrome.Patients and methodsWe presented MDCT imaging findings in three patients with Scimitar syndrome.ResultsMDCT revealed anomalous arterial blood supply from abdominal aorta to right lower lung lobe and anomalous venous drainage into infradiaphragmatic IVC, hypoplastic right pulmonary artery and hypoplastic right lung as well as atrial septal defect and bronchial diverticulum.ConclusionMDCT is essential investigation in Scimitar syndrome.     

Scimitar syndrome – An incidental diagnosis in a case of fibroadenoma

Scimitar syndrome is a rare congenital anomaly which is characterised by anomalous pulmonary venous drainage of the either entire right lung or part of it into the inferior vena cava or portal vein or hepatic vein or right atrium occasionally. This can be associated with hypoplasia of the right lung, dextroposition, underdevelopment of right pulmonary artery and anomalous systemic arterial supply from the descending aorta to the hypoplastic lung. A 36 year old female came with history of swelling in the right breast which turned up to be right breast fibroadenoma. Routine chest radiograph revealed scimitar syndrome which was confirmed on CECT chest.     

Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR

PurposeTo report the anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR.Materials and methodsRetrospective review of the medical records between February 2001 and February 2016 was performed. To identify patients, radiological reports were queried for “Scimitar” or “partial anomalous pulmonary venous return.” Patients with radiological findings of Scimitar syndrome were included. Patients without cross-sectional imaging were excluded. Patients' demographics, radiologic images, and medical notes were reviewed. Two radiologists re-read the available imaging studies. Images were reviewed for Scimitar syndrome confirmation, number and location of Scimitar vein drainage, number of lobes drained by the Scimitar vein, and right pulmonary artery and lung hypoplasia. In addition, the number of pulmonary veins draining into the left atrium, left sided anomalous pulmonary veins, congenital heart disease, aortic arch anomalies, cardiac dextroposition, right ventricular enlargement, pulmonary artery enlargement, and elevated QP:QS ratios were identified. Other associated anomalies including the presence of an anomalous feeding artery and pulmonary sequestration, abnormal lobar pattern, localized bronchiectasis, horseshoe lung, accessory diaphragm, diaphragmatic hernia, vertebral anomalies, and genitourinary tract anomalies were reviewed.ResultsSixteen patients (3 males, 13 females; mean age 39.5 years, range 14 days–72 years) with confirmed Scimitar syndrome on CT and MR imaging were identified. The Scimitar vein drained to the infra-diaphragmatic inferior vena cava (IVC) in ten patients and to the supra-diaphragmatic IVC in six patients. The most common associated anomalies were right ventricle enlargement (93.3%), variant lobar pattern of the right lung (92.9%), enlarged pulmonary arteries (60%), and cardiac dextroposition (50%).ConclusionRecognizing the radiologic characteristics and anatomical associations of Scimitar syndrome is important as features of the primary condition and associated anomalies may have implications in surgical management.     

Embolization of the Systemic Arterial Supply via a Detachable Silicon Balloon in a Child with Scimitar Syndrome

Scimitar syndrome is a rare congenital disorder. It is characterized by partial or total abnormal venous drainage of the right lung into the inferior vena cava, which is often associated with anomalous systemic arterial supply to the right lung, congenital cardiac anomalies, hypoplasia of the right lung and bronchial anomalies. Symptoms depend on the degree of the shunt and severity of the associated anomalies, which determine the treatment. We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. Successful elective surgery was performed 6 months later, in which right pulmonary veins were directed to the left atrium using a Gore-Tex patch by creating an intra-atrial tunnel. The patient has been symptom-free period during 6 months of follow-up, which supports the idea that recurrent pulmonary infections can be eliminated by embolization of the anomalous arterial supply.     

24例镰刀综合征心血管造影征象分析

笔者分析了２４例镰刀综合征心肺血管病变的造影征象及其病理生理意义，并与手术（１４例）和尸解（４例）资料对照。２４例心血管造影示全部或部分右肺静脉异常引流，分别为１７例和７例。异常引流静脉近端局限性狭窄９例，其中２例异常和正常引流静脉间有侧支吻合，右下肺异常体动脉侧支供血１８例。并存各种心脏病变者１８例，马蹄肺６例，右下肺肺隔离症３例，下腔静脉闭锁和狭窄分别为２和１例。右下肺异常体动脉侧支和心脏病变是患者严重症状和肺动脉高压的主要因素。除肺隔离症外，经导管栓塞异常体动脉侧支可使用者明显受益。异常引流静脉狭窄具有限制血液分流的作用。     

Scimitar syndrome associated with gallbladder duplication

Scimitar syndrome is a rare congenital anomaly associated with the venous drainage of the lung to the inferior vena cava through a systemic vein. Chest radiography of an asymptomatic patient, aged 36, showed a linear opacity extending from the pulmonary hilum to the diaphragm, on the right chest (Scimitar sign). Computerized tomography of the thorax exposed that the cause of that image was Scimitar vein. Abdominal ultrasonography revealed duplication in the gallbladder. The literature holds no reports of an association between Scimitar syndrome and gallbladder duplication. Secondary gastrointestinal malformations can also be found in patients suffering from this syndrome.     

Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome

A case in which anomalous systemic venous drainage occurred in association with the hypogenetic lung syndrome (scimitar syndrome) is described. The chest radiograph appearances of the anomalous systemic vein mimicked an anomalous pulmonary or scimitar vein. Angiography demonstrated that the patient also had a small anomalous pulmonary vein draining and a systemic artery supplying, the right lung. As the right lung was hypoplastic, all three features of the hypogenetic lung syndrome were present, in addition to partial anomalous systemic venous drainage.     

Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging

Scimitar syndrome has a variable presentation depending on the age at which the diagnosis is made. We report a case of a young woman (age 18 years) with suspected right pulmonary hypoplasia in whom a scimitar syndrome was diagnosed. Using MRI morphological findings and hemodynamic significance of the syndrome were assessed. Left-to-right shunt was calculated from blood flow measurements performed in the ascending aorta, the main pulmonary artery, and the aberrant scimitar vein.     

Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging

Scimitar syndrome has a variable presentation depending on the age at which the diagnosis is made. We report a case of a young woman (age 18 years) with suspected right pulmonary hypoplasia in whom a scimitar syndrome was diagnosed. Using MRI morphological findings and hemodynamic significance of the syndrome were assessed. Left-to-right shunt was calculated from blood flow measurements performed in the ascending aorta, the main pulmonary artery, and the aberrant scimitar vein.

     

MRI of partial anomalous pulmonary venous return (scimitar syndrome)

We report a case of anomalous pulmonary venous drainage into the inferior vena cava (scimitar syndrome). Cine MRI and 3-D contrast-enhanced MR angiography provides an non-invasive diagnostic technique in the evaluation of anomalous pulmonary venous return.     

Role of multi slice computed tomography in the evaluation of congenital anomalies of tracheobronchial tree and lungs

ObjectiveTo evaluate the role of MSCT in the evaluation of congenital anomalies of tracheobronchial tree and lungPatients and methodsTwenty nine patients with congenital anomalies of tracheobronchial tree and lung were examined using four and six MDCT.ResultsSeven patients (24%) had congenital cystic adenomatoid malformation where successful differentiation of the type was possible. Three patients (10.3%) with bronchopulmonary sequestration were categorized as two (7%) intralobar and one (3.5%) extralobar sequestrations. Four patients (14%) had congenital lobar overinflation. Four patients had Scimitar syndrome with right pulmonary artery hypoplasia, right sided lung hypoplasia, anomalous arterial supply and anomalous pulmonary venous drainage together with pulmonary hypertension. Abnormal bronchial anatomy was revealed in two patients with pulmonary isomerism and one patient with situs inversus. Bronchogenic cyst, tracheal bronchus, Kertagner's syndrome, pulmonary agenesis and horse shoe lung were seen in one patient each. Dynamic MSCT shows the lunate configuration of the trachea during forced expiration in one patient with tracheomalacia. In one patient with tracheo-esophageal fistula, the extent of fistulous tract and the resulting bronchial aspiration were demonstrated.ConclusionMSCT proved to be indispensable as a preoperative planning modality and an essential investigation for congenital anomalies of the lung and tracheobronchial tree.     

Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome

PURPOSE: The authors' goal was to assess the diagnostic accuracy and clinical effect of MRI compared with echocardiography and catheterization in the evaluation of cardiac defects with situs ambiguous. METHOD: Twenty-two patients with visceral heterotaxy syndrome were included. RESULTS: Because situs determined by the relation between the pulmonary artery and bronchi showed most predominantly a tendency toward lateralization, this was regarded as the standard reference of situs determination. For the purpose of this study, patients were classified as having right isomerism (n = 13) or left isomerism groups (n = 9). MRI has several advantages compared with echocardiography or cardiac angiography for examining patients with situs ambiguous. (1) The bronchial, pulmonary arterial, and splenic situs can be readily determined, and discrepancies (n = 2) can be assessed easily. (2) Venoatrial connections are adequately imaged. In particular, all types of total and partial anomalous pulmonary venous return are delineated, regardless of whether restrictions of pulmonary blood flow or pulmonary venous obstructions are involved (n = 4). The courses of vertical veins were easily identified, and the prearterial position was revealed in only one of seven right isomerisms with total anomalous pulmonary venous return. The drain pattern of the hepatic vein can be visualized using three-dimensional spatial information and is useful for total cavopulmonary connection design. (3) Associated complicated cardiac anomalies, particularly the size or peripheral stenosis of the pulmonary arteries, may be evaluated, and this information is useful for palliative shunt operations. CONCLUSION: Because of its wide field of view and imaging, which is not restricted by associated anomalies, a thorough understanding of the cardiovascular anatomy of the situs ambiguous can be achieved using MRI, which is of considerable value in the surgical correction of this complicated anomaly. MRI can obviate or facilitate catheterization in these critically ill patients.     

Acquired anomalous intrapulmonary venous connection secondary to pulmonary venous stenosis

An unusual case of acquired development of anomalous intrapulmonary venous connection with pulmonary venous stenosis is presented. Appearances on a chest radiograph resembled the "scimitar" sign in a patient with previous surgery for partial anomalous pulmonary venous return. Spiral CT and pulmonary arteriography showed stenosis of the right upper pulmonary vein and an anomalous intrapulmonary venous connection between the right upper pulmonary vein and the right lower pulmonary vein. We consider the slow progression of pulmonary vein stenosis led to anomalous intrapulmonary venous connection as an intrapulmonary collateral.     

Horseshoe lung: clinical, pathologic, and radiologic features and a new plain film finding

Horseshoe lung is a rare congenital malformation in which an isthmus of pulmonary parenchyma extends from the right lung base across the midline behind the pericardium and fuses with the base of the left lung. Six cases are presented and eight previously published case reports are reviewed. Eleven of these 14 cases occur in conjunction with scimitar syndrome, the complex of anomalies including hypoplasia of the right lung, anomalous right pulmonary venous return, and anomalous arterial supply to the right lung. Differentiation of scimitar syndrome from horseshoe lung can be made on the plain chest film, if, in addition to the typical radiographic findings of scimitar syndrome, there is evidence of a fine linear density in the medial aspect of the left base representing the lateral extent of the pulmonary isthmus. Recognition of horseshoe lung is important because children present in the first few years of life with significant respiratory symptoms, whereas the scimitar syndrome alone is usually discovered fortuitously after the first decade of life.     

Partial anomalous pulmonary venous return in Turner syndrome

PurposeThe aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome.Methods and resultsAll Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described. Partial anomalous pulmonary venous return was diagnosed in 24 (25%) out of 96 Turner patients included and 14 (58%) of these 24 partial anomalous pulmonary venous return had not been reported previously. Right atrial or ventricular dilatation was present in 11 (46%) of 24 partial anomalous pulmonary venous return patients.ConclusionWhen studied with advanced imaging modalities and looked for with specific attention, PAPVR is found in 1 out of 4 Turner patients. Half of these patients had right atrial and/or ventricular dilatation. Evaluation of pulmonary venous return should be included in the standard protocol in all Turner patients.     

Anomalous origin of the right pulmonary artery from the ascending aorta: Diagnosis by magnetic resonance imaging

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital anomaly. Magnetic resonance imaging (MRI) was performed on three patients with anomalous origin of the right pulmonary artery from the ascending aorta. ECG-gated, Tlweighted, spin-echo MRIs and cine MRIs were obtained. In one patient, postoperative MRI was also obtained. Echocardiography and cardiac catheterization were performed in three patients and angiocardiography was performed in two. MRI clearly showed anomalous origin of the right pulmonary artery from the posterior aspect of the ascending aorta, as well as combined anomalies including patent ductus arteriosus, aortopulmonary window, and interruption of the aortic arch in all three patients. Echocardiography missed this anomaly in all three. We suggest that MRI is an accurate imaging modality in diagnosing anomalous origin of the right pulmonary artery from the ascending aorta, obviating the need to perform angiocardiography     

二尖瓣闭锁的MRI诊断

目的 分析二尖瓣闭锁（MA）的病理改变和MRI表现,方法 回顾分析5例经手术证实MA的MRI表现,并与心血管造影（CAG）比较,结果 显示了3型MA的病理改变：双心室左侧房室瓣无孔1例,右室型单心室左侧房室无连接3例,以及左室型单心室左侧房室瓣无孔1例,主要合并畸形有：房间隔膨出瘤1例,全肺静脉异位引流心上型1例,左肺动脉缺如1例及肺动脉狭窄4例,5例行右室和（或）右房造影,除1例外均难以显示MA解剖结构,结论 MRI在MA的诊断中有一定的临床应用价值。     

电子束CT在肺静异常连接诊断中的临床价值

目的 探讨电子束CT（EBCT）在肺静脉异常连接诊断中的临床价值。材料与方法 EBCT诊断肺静脉异常连接患者21例,其中男13例,女8例。年龄3－63岁,平均15.6岁。17例行增强单层容积扫描,4例行增强连续容积扫描。全部病例均行表面阴影显示法三维重建。17例有心血管造影或手术结果,其中9例单纯行造影,4例单纯行手术,4例造影后手术。结果 20例术前资料,EBCT诊断为完全型肺静脉异常连接12例,部分型肺静脉异常连接8例,前者包括心上型3例,心脏型8例和混合型1例。EBCT均清晰显示异常连接肺静脉的数目、分布和位置。1例术后复查病例,ERCT示吻合口狭窄。7例完全型肺静脉异常连接者,EBCT提示合并内脏心房异位综合征。8例有手术对照者,EBCT对肺静脉异常连接的定性、定位诊断全部正确。结论 EBCT及其三维重建可准确显示异常连接 肺静脉的位置、数目和术后狭窄,对明确胸腹并发畸形有明显优势,是一种重要的无创性诊断肺静脉异常连接的方法。     

Partial anomalous pulmonary venous return

Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that occurs in 0.4 to 0.7% of postmortem examinations. Ninety percent of these anomalies are associated with an atrial septal defect. Partial anomalous pulmonary venous return occurs more commonly on the right than the left and is manifested by abnormal return of the pulmonary veins to the central venous circulation. Most patients are asymptomatic, but when symptoms are present they are due to shunting of oxygenated blood to the venous circulation. We submit the case of a recently activated solider who presented with dyspnea on exertion refractory to inhaled corticosteroids and an 8.5-mm solitary pulmonary nodule. Further diagnostic imaging revealed PAPVR. Our case appears to be the first report of a solitary pulmonary nodule as the initial presentation of a right upper lobe PAPVR with return to the superior vena cava in the absence of associated atrial septal defect.     

MR imaging of partial anomalous pulmonary venous connections

Magnetic resonance imaging was performed on 11 patients with partial anomalous pulmonary venous connections (PAPVC). Ten of these patients also had echocardiographic examinations, eight of which included color-flow Doppler studies. The diagnosis of PAPVC was confirmed in each of these patients by surgery or angiography. Fourteen anomalous pulmonary venous connections were identified, 10 involving the right upper lobe pulmonary vein and 4 involving the left upper lobe pulmonary vein. This retrospective review demonstrated that all 14 anomalous venous connections were correctly identified by MR imaging, whereas only 8 of 13 (62%) were identified by echocardiography. With MR, 89% of all the pulmonary veins and 93% of the anomalous pulmonary veins were visualized on axial images, while 41% of all pulmonary veins and 71% of anomalous veins were seen on coronal MR images. There were five atrial septal defects (ASDs), four of the sinus venous type and one of the septum secundum type. All five ASDs were correctly identified with MR imaging; three of four ASDs were identified with echocardiography. We conclude that MR imaging provides an accurate noninvasive method of depicting the anatomic abnormalities associated with PAPVC.