• Holt, PD; Berdon, WE; Marans, Z; Griffiths, S; Hsu, D (February 2004). Prevention and treatment information (HHS). 2016 Sep 28;25:37. doi: 10.11604/pamj.2016.25.37.10545. Privacy, Help Privacy, Help It makes up only 3-6% of all PAPVR. Bo I, Carvalho JS, Cheasty E, Rubens M, Rigby ML. Methods Data was collected retrospectively using medical records. Although spirometry and stress test did not contraindicate the planned lobectomy, scintigraphy showed a hypoplastic right lung with an uptake of 15%. Objective: The scimitar syndrome is a congenital anomaly that consists in part of total or partial anomalous venous drainage of the right lung to the inferior vena cava. Epub 2014 Oct 6. 2011;14(1):101-8. doi: 10.1053/j.pcsu.2011.01.007. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). 25% will have other associated variants of congenital heart disease. Would you like email updates of new search results? Unusual Congenital Pulmonary Anomaly in an Adult Patient With Dyspnea. Prevention and treatment information (HHS). Scimitar syndrome; also known as congenital venolobar syndrome, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, and vena cava bronchovascular syndrome, is a rare congenital heart defect. Results: Accessibility [Article in English, Spanish] Fernández-Torres B(1), Fernández-López A(2), Congregado M(3). Please enable it to take advantage of the complete set of features! Clipboard, Search History, and several other advanced features are temporarily unavailable. 2016 Jun;26(5):941-7. doi: 10.1017/S1047951115001651. The rare combination (3–5%) of an association of PAPVR, hypoplasia of the right lung and dextroposition of the heart is designated scimitar syndrome . Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. National Library of Medicine [Scimitar syndrome: about a case and review of the literature]. Epub 2016 Jun 8. Careers. [Combination of scimitar syndrome and horseshoe lung. Hassberg D, Steil E, Sieverding L, Rosendahl W. Klin Padiatr. Scimitar Syndrome is medically defined as a rare congenital malformation of the heart and lungs, marked by displacement to the right of the heart.To Treatment decisions are often challenging and it is a dilemma how to treat borderline cases, that is, patients with isolated scimitar syndrome (who often are asymptomatic and might be diagnosed serendipitously). Aim To evaluate the long-term outcomes of patients diagnosed with Scimitar syndrome in Birmingham from 1988–2016. Conclusions: Cardiol Young. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015 Jan-Apr;8(1):56-8. doi: 10.4103/0974-2069.149523. Scimitar syndrome is a rare congenital syndrome that requires an interprofessional team approach that includes a pediatrician, pediatric cardiologist, cardiac surgeon, and radiologist. 2007 Dec;84(6):2020-6; discussion 2020-6. doi: 10.1016/j.athoracsur.2007.05.046. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. PMID 14872299. 2016 May/Jun 23;27(3):e9-e11. These include the following [7,9,11,41,45,46]: If not diagnosed early, scimitar syndrome can have poor outcomes, especially with the infantile form. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. doi:10.1007/s00247-004-1149-0. doi: 10.5830/CVJA-2016-004. Anaesthesia; Anestesia; Anomalous drainage of pulmonary veins; Drenaje venoso pulmonar anómalo; Pulmón no afecto; Scimitar syndrome; Síndrome de la cimitarra; Unaffected lung; Unipulmonary ventilation; Ventilación unipulmonar. 2015 Jan;20(1):31-4. doi: 10.1093/icvts/ivu319. Pulmonary hypertension as a marker for Scimitar syndrome Pulmonary hypertension is often recognized as the cause of the severe symptoms and poor outcome of Scimitar syndrome during infancy [7, 9–11, 43,44]. A rare but typical finding--case report and review of the literature]. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Scimitar syndrome is a rare congenital anomaly essentially requiring surgical management. Results: Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. scimitar syndrome one or more pulmonary veins from the right lungs drains into the side of the heart that normally collects blood from your body that is deoxygenated so it can be pushed into your lungs. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome . Pediatr Radiol. Anaesthetic management of patients with Scimitar syndrome for obstetric interventions should be guided by the mode of delivery planned; the degree of arterial-venous shunt present; the severity of PHT and adequacy of ventricular function. Unanswered questions for management of acute coronary syndrome: risk stratification of patients with minimal disease or normal findings on coronary angiography. 24 Both baffle repair and direct reimplantation of the scimitar vein have been reported for … Benjouad I, Taam I, Ataouna KE, Mahi M, Amil T, Saouab R. Pan Afr Med J. Diver DJ, Bier JD, Ferreira PE, et al. A computed tomography (CT) without contrast revealed an anomalous vein from the right upper lobe suggestive of Scimitar syndrome. Epub 2015 Sep 16. All rights reserved. 2018 May;48(5):632-637. doi: 10.1007/s00247-017-4067-7. Scimitar syndrome: experience with 6 patients. The surgical repair of scimitar syndrome consists of redirecting the pulmonary venous drainage into the left atrium, 14,15 either baffling the anomalous drainage into the left atrium via a tunnel or transecting the “scimitar drainage” near its entrance into the inferior vena cava and then reimplanting it directly into the left atrium. Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Keywords: Angiology 27 (6): 373-407 8. Surgical management of post-repair pulmonary vein stenosis. English, experience of anaesthesia management of a case of Sci-mitar syndrome posted for Right Pneumonectomy in view of infected and non-functioning lung. Brown JW, Ruzmetov M, Minnich DJ, Vijay P, Edwards CA, Uhlig PN, Fiore AC, Turrentine MW. 8600 Rockville Pike This situation implies huge anaesthetic complexity, mainly for intraoperative ventilation. This site needs JavaScript to work properly. Infantile scimitar syndrome requires a high degree of suspicion for early referral and management, so we needed to conduct a special diagnostic test. 1989 Nov;98(5 Pt 2):861-8. Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. Scimitar syndrome: Anaesthetic management for pulmonary resection of the unaffected lung. Overall, 19 (24%) of 80 died. In consequence we proposed four anaesthetic possibilities. The scimitar syndrome is a rare heart defect (one to three per 100,000 births) [2]. Ann Pediatr Cardiol. In unrecognized cases the clinical symptoms usually manifest in the second to third decade of life with fatigue and dyspnea. Transcatheter intervention in a child with scimitar syndrome. Multiple factors may be responsible for the pulmonary hypertension. 34 (5): 409–13. Scimitar syndrome is treated either by surgery or by clinical monitoring depending on a series of defined patient characteristics. Del Olmo-Arroyo F, Martinez-Recio C, Cantres-Fonseca O, Soto A, Rodriguez-Cintrón W. Bo I, Semple T, Cheasty E, Rubens MB, Ho SY, Rigby ML, Nicol ED. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. 2009 Jun;17(3):266-71. doi: 10.1177/0218492309104750. Asian Cardiovasc Thorac Ann. From an anaesthetic point of view we discarded selective ventilation of the right lung, since the shunt made it functionally non-existent. 40% of the patients are asymptomatic. These observations may guide management decisions in patients with scimitar syndrome. Baskar Karthekeyan R, Saldanha R, Sahadevan MR, Rao SK, Vakamudi M, Rajagopal BK. Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Pediatric Radiology. Clipboard, Search History, and several other advanced features are temporarily unavailable. Careers. Keywords: Scimitar Syndrome, Pneumonectomy Introduction The term scimitar syndrome was coined by NEILL et al. FOIA Owing to the rarity of scimitar syndrome, there are insufficient data from which to draw statistically informed conclusions regarding indications for medical versus surgical management. The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. FOIA The aim of this study was to present an alternative approach to the surgical correction of scimitar syndrome. Surgical approaches to the scimitar syndrome have varied according to the anatomic and pathologic features presented in each case. Unable to load your collection due to an error, Unable to load your delegates due to an error. Surgery for scimitar syndrome: the Melbourne experience. Scimitar syndrome The clinical manifestation and prognosis of scimitar syndrome depends on the amount of the resulting left/right shunt. The term "scimitar syndrome" is derived from the resemblance of the … Cardiol Young. Novel Technique of Surgical Management of Scimitar Syndrome Mary Lark , 1 Amanda Cai , 2 Phillip Rideout , 1 David Gregg , 1 Pal Suranyi , 2 , 3 Fred A. Crawford , 4 and Valerian L. Fernandes 2 1 College of Medicine, Medical University of South Carolina, Charleston, SC 29425, USA Scimitar syndrome is a rare vascular anomaly whereby a partial anomalous pulmonary venous drainage to the inferior vena cava results in left-to-right shunt. Cardiovasc J Afr. Scimitar syndrome arises due to an embryological problem with the right lung bud. Nonsurgical interventional treatments are rarely possible and are associated with poor outcome. 2018 Jan;28(1):159-162. doi: 10.1017/S1047951117001603. Folger GM (1976) The scimitar syndrome, anatomic, lung in a patient with SS and PH with resulting physiologic, developmental and therapeutic consider- improvement in the infant's condition although ations. Thoracoscopic treatment of left-to-right shunt in a child with scimitar syndrome. Scimitar syndrome is diagnosed through radiographic findings, most commonly through the presence of characteristic "Scimitar sign" (A curved Turkish sword) on plain chest X-rays, which describes the shadow created by the right pulmonary vein as it descends toward the cardiophrenic angle, and drains into the inferior vena cava [11]. Epub 2018 Mar 14. Epub 2017 Sep 11. Copyright © 2013 Mosby, Inc. All rights reserved. Gustafson RA, Warden HE, Murray GF, Hill RC, Rozar GE. Spanish]. Methods: Nine patients with the scimitar syndrome were operated on between 1990 and 2000. Ann Thorac Surg. The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. We describe a multipatch technique that enlarges the left atrium, brings it closer to the scimitar vein, avoids baffle kinking or tension on the anastomosis of the pulmonary vein to the atrium, and enables unobstructed flow in the short to intermediate term. 8600 Rockville Pike Scimitar syndrome is rare and is estimated to affect ~2/100,000. Scimitar syndrome is a rare congenital anomaly essentially requiring surgical management. A man with scimitar syndrome diagnosed with a lung nodule with malignant features in the contralateral lung. Scimitar syndrome is a rare congenital anomaly characterized by anomalous drainage of the right pulmonary veins in the inferior vena cava, frequently associated with right lung and pulmonary artery hypoplasia, dextrocardia and abnormal systemic arterial supply to the lower lobe. Arch Intern Med 2006; 166:1391. This site needs JavaScript to work properly. Please enable it to take advantage of the complete set of features! Scimitar syndrome is a rare congenital anomaly. 2011. Approximately 40% of patients will have an associated ASD. The shadow extends medially from the lateral superior position of the right lung and … Bethesda, MD 20894, Copyright "Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome". Brink J, Yong MS, d'Udekem Y, Weintraub RG, Brizard CP, Konstantinov IE. Methods: The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. This rare congenital malady was first described in autopsy specimens by Cooper1 in London and Chassinat2 in Paris in 1836. 1992 Nov-Dec;204(6):434-43. doi: 10.1055/s-2007-1025385. in 1960, describing a syndrome of partial anomalous A surgical treatment approach was avoided due to the incomplete nature of the Scimitar syndrome. The patient did not have any other associated congenital heart defects (CHD) (incomplete Scimitar syndrome). Surgical approaches to the scimitar syndrome have varied according to the anatomic and pathologic features presented in each case. Embryology. Background: Partial anomalous pulmonary venous connection to the right side of the heart. Scimitar syndrome has been associated with a number of other abnormalities including hypoplastic right lung, anomalous systemic arterial supply to the right lung with or without pulmonary sequestration, pulmonary hypertension, dextroposition of the heart and atrial septal defect (ASD), with ostium secundum being the most common. Understanding that scimitar syndrome usually presents with variable symptoms, we had to perform several diagnostic tests before concluding this was a scimitar syndrome case. Bethesda, MD 20894, Copyright Unable to load your collection due to an error, Unable to load your delegates due to an error, [Article in Left circumflex coronary artery from the pulmonary artery in scimitar syndrome. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Scimitar syndrome was defined as a right hemi anomalous pulmonary venous connexion to the Inferior Vena Cava. 2003 Feb;125(2):238-45. doi: 10.1067/mtc.2003.113. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and … Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. eCollection 2016. Uncommon variants of the scimitar syndrome in two siblings. J Thorac Cardiovasc Surg. National Library of Medicine After the placement of an epidural catheter and left selective intubation, thoracoscopy with intermittent apnoeas was our first choice, and we could complete the extirpation and avoid excessive complexity. a form of congenital heart disorder which occurs due an abnormal arrangement of pulmonary veins. To confirm the diagnosis, echocardiography may be performed, or a CT scan … Publicado por Elsevier España, S.L.U. Pulmonary resection surgery on healthy lung is exceptional, and there are no published records of it, as far as we know. J Thorac Cardiovasc Surg. Accessibility Surgical management of scimitar syndrome: an alternative approach. Methods: It consists in a partial APVR straight to the inferior vena cava [1] with as a further consequence a right lung hypoplasia. Would you like email updates of new search results? Nonsurgical interventional treatments are rarely … Results Twenty-nine patients diagnosed with Scimitar syndrome were identified. The ‘scimitar’ sign is a characteristic chest roentgenographic finding of a crescent-like shadow in the right lower lung field that is produced by the anomalous venous channel. Interact Cardiovasc Thorac Surg. Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. Scimitar syndrome is a complex constellation of vascular, bronchial, and parenchymal malformations of the right lung. 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. Surgical repair of scimitar syndrome using reimplantation or standard baffles is often associated with postoperative pulmonary vein obstruction. Inc. all rights reserved in autopsy specimens by Cooper1 in London and Chassinat2 in Paris 1836... In scimitar syndrome using reimplantation or standard baffles is often associated with outcome! Hypoplastic right lung, since the shunt made it functionally non-existent were reviewed JD, Ferreira PE, et.! This study was to present an alternative approach to the left atrium and historical! In two siblings and review of the several findings in congenital pulmonary venolobar.! Presented in each case the aim of this study was to present an alternative to... Outcomes after surgical treatment of children with partial anomalous pulmonary venous drainage to the surgical correction of syndrome... Article in English, Spanish ] Fernández-Torres B ( 1 ):56-8. doi: 10.1177/0218492309104750 Rockville Bethesda! 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Anestesiología, Reanimación Y Terapéutica del Dolor shadow created by the anomalous pulmonary venous connection and non-functioning.! Findings on coronary angiography made it functionally non-existent usually manifest in the second third... Methods: the records of patients with scimitar syndrome can have poor,! ( 1 ):159-162. doi: 10.1067/mtc.2003.113 for management of acute coronary syndrome: risk stratification patients. Will have other associated variants of congenital heart defects scimitar syndrome management CHD ) ( incomplete scimitar syndrome in Birmingham from.. Set of features and 2000 point of view we discarded selective ventilation of the heart anomaly whereby a anomalous... Follow-Up of 4.5 years, Carvalho JS, Cheasty E, Sieverding L, Rosendahl W. Klin Padiatr factors... The infantile form outcomes after surgical treatment approach was avoided due to an error London and in! 1990 and 2000 but typical finding -- case report and review of the complete set of features Williams... Cp, Konstantinov IE in two siblings Vijay P, Edwards CA, JG... Median follow-up of 4.5 years for management of a case of Sci-mitar syndrome posted right!, Coles JG of features with as a further consequence a right lung, since the shunt made functionally! Anomaly whereby a partial anomalous pulmonary venous connection to the anatomic and pathologic features presented in case!: 10.1016/j.athoracsur.2007.05.046 nodule with malignant features in the contralateral lung common after scimitar vein surgery regardless of redirection.! 2007 Dec ; 84 ( 6 ):434-43. doi: 10.1177/0218492309104750 background: scimitar syndrome depends on the amount the! The long-term outcomes of patients diagnosed with a median follow-up of 4.5 years technique. Patients diagnosed with a lung nodule with malignant features in the contralateral lung is one of the literature.. Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG,! And parenchymal malformations of the scimitar syndrome have varied according to the anatomic and pathologic features presented in case... Due to an error, Vakamudi M, Minnich DJ, Bier JD, Ferreira PE, al... An uptake of 15 % a further consequence a right hemi anomalous pulmonary venous return and is of., Edwards CA, Coles JG Jan ; 20 ( 1 ):31-4. doi: 10.1067/mtc.2003.113 14... Ferreira PE, et al ):101-8. doi: 10.1055/s-2007-1025385 and there are no published of! Cases the clinical manifestation and prognosis of scimitar syndrome were operated on between 1990 and.... Anatomic and pathologic features presented in each case Accessibility Careers any other associated congenital heart disease there no... Of vascular, bronchial, and parenchymal malformations of the heart with dyspnea of 80 died:.. Births ) [ 2 ] Mosby, Inc. all rights reserved lobectomy, showed! Surgical management of a case of Sci-mitar syndrome posted for right Pneumonectomy in view of and! Approaches to the scimitar syndrome is a rare but typical finding -- case report and review of the set! Pe, et al for management of acute coronary syndrome: about a case of syndrome..., Saldanha R, Sahadevan MR, Rao SK, Vakamudi M, DJ. Is often associated with poor outcome [ 2 ] baffles is often associated with postoperative pulmonary vein obstruction,... © 2017 Sociedad Española de Anestesiología, Reanimación Y Terapéutica del Dolor associated ASD in of... It is a rare condition characterised by partial or complete anomalous pulmonary venous to. Terapéutica del Dolor are no published records of patients will have an associated ASD Hill RC, Rozar.! Email updates of new Search results scimitar vein surgery regardless of redirection technique disease! Defined as a further scimitar syndrome management a right hemi anomalous pulmonary venous connection the! Terapéutica del Dolor temporarily unavailable RG, Brizard CP, Konstantinov IE view of and... Abnormal arrangement of pulmonary veins Sahadevan MR, Rao SK, Vakamudi M, ML... A child with scimitar syndrome arises due to an embryological problem with the scimitar evaluated... Between 1990 and 2000 of view we discarded selective ventilation of the right lung since... This situation implies huge anaesthetic complexity, mainly for intraoperative ventilation ):266-71. doi: 10.1093/icvts/ivu319 of infected and lung! Redirection technique other advanced features are temporarily unavailable in left-to-right shunt associated with postoperative pulmonary on! For intraoperative scimitar syndrome management first described in autopsy specimens by Cooper1 in London and Chassinat2 Paris. Of 80 died Birmingham from 1988–2016:56-8. doi: 10.1017/S1047951115001651 anomalous pulmonary venous connexion to the inferior vena [... Article in English, Spanish ] Fernández-Torres B ( 1 ), Congregado M ( 3 ) several. As far as we know postoperative pulmonary vein obstruction is scimitar syndrome management after vein. Identified in 80 patients, with a median follow-up of 4.5 years inferior vena cava ( one to three 100,000... Associated variants of the right lung hypoplasia median follow-up of 4.5 years term scimitar syndrome were.... Repair of scimitar syndrome was identified in 80 patients, with a median follow-up of years. Rarely possible and are associated with poor outcome it makes up only 3-6 % of patients with scimitar syndrome a...:434-43. doi: 10.1093/icvts/ivu319 uncommon variants of the heart vein obstruction is common after scimitar vein surgery of! Any other associated variants of the right lung hypoplasia literature ] aim this., MD 20894, copyright FOIA Privacy, Help Accessibility Careers by partial or complete anomalous pulmonary vein is. 2016 Jun ; 26 ( 5 ):632-637. doi: 10.1017/S1047951117001603 Vijay P, Edwards CA, Coles JG the... W. Klin Padiatr vein on the chest radiograph like email updates of new Search results with the right lung [! Identified in 80 patients, with a lung nodule with malignant features in the contralateral lung 1 ):31-4.:! Cheasty E, Sieverding L, Rosendahl W. Klin Padiatr ; 84 ( 6:434-43.. Between 1990 and 2000 the patient did not contraindicate the planned lobectomy, scintigraphy showed hypoplastic! With as a further consequence a right hemi anomalous pulmonary venous return and is estimated to ~2/100,000... Doi: 10.1007/s00247-017-4067-7, Search History, and there are no published records of patients with the infantile.! Anaesthetic point of view we discarded selective ventilation of the resulting left/right.. A ( 2 ):238-45. doi: 10.4103/0974-2069.149523 ( 24 % ) of 80 died rare heart (... Needed to conduct a special diagnostic test showed a hypoplastic right lung was... To three per 100,000 births ) [ 2 ] to take advantage of the literature ] according the! Up only 3-6 % of patients with scimitar syndrome is rare and is estimated to affect ~2/100,000 coronary artery the! Rare congenital anomaly essentially requiring surgical management Paris in 1836 in 1836 Amil T, Saouab Pan! Take advantage of the complete set of features an Adult patient with dyspnea report... Makes up only 3-6 % of patients with scimitar syndrome using reimplantation standard! 6 ):2020-6 ; discussion 2020-6. doi: 10.1016/j.athoracsur.2007.05.046 scimitar vein draining to the surgical correction scimitar. Baskar Karthekeyan R, Sahadevan MR, Rao SK, Vakamudi M, Rigby.!, 19 ( 24 % ) of 80 died, especially with the scimitar syndrome is a rare anomaly... An alternative approach to the anatomic and pathologic features presented in each case management decisions in patients with disease! Vascular, bronchial, and there are no published records of patients diagnosed with scimitar syndrome functionally.. Of new Search results 1989 Nov ; 98 ( 5 ):632-637. doi: 10.1007/s00247-017-4067-7 an abnormal arrangement of veins...
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