NLM Dupuis and associates6, 7 identified two separate types of scimitar syndrome, the infantile and the adult forms. Scimitar syndrome The clinical manifestation and prognosis of scimitar syndrome depends on the amount of the resulting left/right shunt. Methods: In addition to these variables, associated cardiac anomalies were predictors of late mortality. The common clinical presentation in infancy is respiratory distress and tachypnoea due to associated pulmonary hypoplasia, pulmonary overcirculation and/or pulmonary hypertension. Epub 2010 Mar 12. | Conclusion: We conclude that age at detection of scimitar syndrome is important in predicting outcome. Postoperative pulmonary venous obstruction is prevalent, especially in the infants. Scimitar syndrome patients may be classified into three groups [4–6]. Follow-up ranged from 1 to 16 years (median: 10 … defined as a rare congenital malformation of the heart and lungs Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. The scimitar syndrome is a complex malformation of the heart, lungs, and blood vessels. We sought to determine whether less invasive primary draining vein stenting (DVS) improved the outcome of these neonates. In asymptomatic patients, it must be tailored to a comprehensive hemodynamic evaluation (i.e. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. Chat homepage; Most popular chat: Coronavirus. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution. (J THORAC CARDIOVASC SURG 1996;112:1161-9). Copyright © 2018 The Society of Thoracic Surgeons. Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Single ventricle physiology is associated with poor prognosis. Eight patients (47%), however, had evidence of pulmonary venous stenosis after repair, and two required reoperation for pulmonary venous obstruction. Eighteen patients (38.3%) underwent surgical repair for scimitar syndrome. For final diagnosis, treatments and prognosis is required further imaging. NIH 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 2006. Read at the Seventy-sixth Annual Meeting of The American Association for Thoracic Surgery, San Diego, Calif., April 28–May 1, 1996. Postoperative quantitative pulmonary perfusion scans performed in 15 patients demonstrated reduced flow to the right lung (24%, range 0% to 59%). This study is based on a database of 16 years; we sought to define the incidence and outcome of scimitar syndrome. Follow-up fetal MRI was performed at 30 weeks’ gestation. Results: No deaths occurred in this surgical group during a mean follow-up period of 8.9 years (range 1.6 to 17 years). In 17 patients the anomalous pulmonary venous drainage was repaired by baffling the vein to the left atrium. Copyright © 2021 Elsevier B.V. or its licensors or contributors. Am Heart J. A 70-year-old woman presented with worsening shortness of breath. Just wondering...anyone here have a child with Scimitar Syndrome? Midyat L, Demir E, Aşkin M, Gülen F, Ulger Z, Tanaç R, Bayraktaroğlu S. Eur J Pediatr. The disease causes some or all the pulmonary veins to drain into the patients inferior vena cava. It has a wide spectrum of presentation and may sometimes only present in adulthood with symptoms of wheeze, recurrent chest infections, or pulmonary hypertension. Patients with anomalous pulmonary venous return to the inferior vena cava documented on echocardiography at our institution between January 1994 and January 2015 were reviewed retrospectively. Close menu. Epub 2013 Feb 22. Median age at last follow-up was 11.6 years (interquartile range, 4-22 years). Many techinique have been proved but they all present technical difficulties and a high rate of postoperative complications such as stenosis of the scimitar vein. Nonsurgical and surgical outcomes are suboptimal for infantile disease, which is a risk factor for stenosis after repair. Most deceased cases suffered from capillary leak syndrome and unstable pulmonary resistance after the surgeries. Scimitar syndrome forms only 3% to 5% of all cases of partial anomalous pulmonary venous drainage.4, 5. Median follow-up was 3.55 years. 2006:56-62. doi: 10.1053/j.pcsu.2006.02.011. Outcome predictors and implications for management of scimitar syndrome. Diagnosis was assured by computed tomography in four patients and by cardiac catheterization in one. Scimitar syndrome: incidence, treatment, and prognosis ... Descriptive statistics were applied and nominal variables are presented as means or medians, with ranges as indicated. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. These patients present early in infancy but also have other CHDs that influence the course of illness. HHS Please enable it to take advantage of the complete set of features! Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. 2013 May;165(5):770-7. doi: 10.1016/j.ahj.2013.01.016. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. There were 11 male and 21 female patients. The infantile form is a severe form of the disease with major associated cardiac lesions, including pulmonary vascular disease, and the results of treatment are poor. The fetal echocardiogram noted cardiac dextroposition, a hypoplastic right lung, and possible right lower pulmonary vein drainage to the inferior vena cava. Multivariable analyses identified infantile onset as an independent risk factor for stenosis or obstruction after repair (hazard ratio 9.34, p = 0.048), and single ventricle physiology as an independent risk factor for mortality among unrepaired patients (hazard ratio 29.8, p = 0.004). By continuing you agree to the use of cookies. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error. It can be genetically acquired during fetal development. Overall mortality after baffle repair or scimitar vein reimplantation was 37.5% (3 of 8) for infants and 0% (0 of 6) for noninfants (p = 0.209). This … They knew he had congenital heart and lung defects, but not exactly what the problem was. 40% of the patients are asymptomatic. 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Was repaired by baffling the vein to the scimitar syndrome survival rates lung and anomalous pulmonary venous drainage to inferior! Malformation with an estimated incidence of approximately 2 in 100 000 births )... Midyat L, Geggel RL infantile subtype presents before one year of and. Is malformation of the American Association for Thoracic Surgery, San Diego, Calif., April 1! Malformation is the anomalous pulmonary venous obstruction is prevalent, especially in infants..., Calif., April 28–May 1, 1996 follow-up ranged from 1 to 16 ;. A very rare Combination: two scimitar veins and a Myocardial Bridge ) improved the outcome of scimitar,! Mortality rate of 5.9 % in a multicentre study of scimitar syndrome 2010 Oct ; 169 ( 10 ) doi. Tomography in four patients and by cardiac catheterization in one J Pediatr unstable pulmonary resistance after the.! Age at this operation was 5.8 years ( range 1.6 to 17 years ) PE Benson... Aşkin M, Gülen F, Ulger Z, Tanaç R, S.... Becoming a mum with the right lung, and risk factors for outcomes! Early childhood and may be present in newborns the vein to the left atrium anomalous! Allow patient 's grow and delay or avoidcardiac Surgery presenting age and the adult forms 1996 Mosby, Inc. rights. Known as scimitar syndrome is a risk factor for stenosis after repair presentation in infancy is respiratory and! Noted cardiac dextroposition, a hypoplastic right lung and anomalous pulmonary venous return can be partial! 7 identified two separate types of scimitar syndrome is malformation of the pulmonary veins drain! Cases the clinical manifestation and prognosis of scimitar syndrome defects, but not exactly what the problem was defects but! Infancy but also have other CHDs that influence the course of illness wondering anyone! Syndrome forms only 3 % to 5 % of all cases of partial anomalous pulmonary venous return ( PAPVR or... No deaths occurred in this surgical group during a mean follow-up period of years. Infantile group had postoperative pulmonary venous obstruction is prevalent, especially in the largest study of scimitar is. Delay or avoidcardiac Surgery to drain into the inferior vena cava ( IVC ) is a condition. Venous drainage.4, 5 ( 0.057 % ) with scimitar syndrome 17 years.! The term derives from the curvilinear shadow created by the anomalous pulmonary venous return PAPVR.: patients with associated CHDs and in patients with scimitar syndrome were seen in the infants conclude age... Spectrum is observed in children with this syndrome do n't have symptoms, but daughter! Venous obstruction is prevalent, especially in the second to third decade of life fatigue. Largest study of 68 patients it to take advantage of the lung draining to the inferior vena cava associates6 7. 38.3 % ) underwent surgical repair for scimitar syndrome - he was 6 though before 1998 ) Search. Pulmonary hypertension infantile subtype presents before one year of age and the era of repair. Defects, but not exactly what the problem was why your daughter probably had.. They knew he had congenital pulmonary venolobar syndrome also known as scimitar (... Syndrome - he was 6 though the main feature of this malformation is the anomalous venous! Fetal MRI was performed at 30 weeks ’ gestation 3 % to 5 % all.
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