Scimitar Syndrome in Children and Adults: Natural History, Outcomes, and Risk Analysis

Wang, Hanjay; Kalfa, David; Rosenbaum, Marlon; Ginns, Jonathan; Lewis, Matthew J.; Glickstein, Julie S.; Bacha, Emile A.; Chai, Paul J.

doi:10.1016/j.athoracsur.2017.06.061

Cited by 55 publications

(85 citation statements)

References 17 publications

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“…It can be associated with other congenital heart defects such as single ventricular defect causing congestive cardiac failure [4]. The atrial septal defects are also noted to coexist with the scimitar syndrome [5].…”

Section: Discussionmentioning

confidence: 99%

“…Embolization of the aortopulmonary collaterals can also be done by detachable coils [11]. During our literature search, we came across other case reports of scimitar syndrome associated with other congenital defects such as VACTERL, H-type tracheoesophageal fistula [5,12], and imperforated anus [13]. We also came across case reports showing features of failure to thrive due to recurrent chest infections, leading to immediate repair of the defect.…”

Section: Discussionmentioning

confidence: 99%

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Scimitar syndrome – Uncommon variant with common presentation

Dave¹,

Parikh²,

Vasoya³

2020

IJCR

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S cimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature of partial or total anomalous pulmonary venous drainage of the right lung to the systemic circulation [1]. It is characterized by partial anomalous pulmonary venous drainage of the right lung causing left-to-right shunt along with the right lung hypoplasia, dextraposition of the heart [2], and pulmonary hypertension due to aortopulmonary collaterals [3]. When visualized on imaging, it resembles a backsword with a curved blade, also known as scimitar in Middle East [4]. In infants, it can present as recurrent lung infection and can develop complications such as pulmonary arterial hypertension and congestive cardiac failure [5], while in adults, it usually remains asymptomatic [6]. It can be associated with other congenital heart defects such as single ventricular defect causing congestive cardiac failure [7] or atrial septal defects [8]. The surgical treatment includes rightsided pneumonectomy [9] or, a tunnel connecting the scimitar vein, and the left atrium can be made by Dacron graft through intra-atrial baffle technique [10]. CASE REPORT A 9-month-old male baby presented to the pediatric outpatient clinic with complaints of fever with cough, cold, and decreased oral intake for 5 days. His vitals demonstrated low-grade fever of 98.2°F with a pulse rate of 110/min, and respiratory rate of 36/ min. On physical examination, he looked pale and crepitations were heard over his chest. Therefore, he was admitted and commenced on IV antibiotics and nebulization. The laboratory findings showed anemia (6.70 g/dl), leukocytosis with counts of 13,200/mm 3 , and elevated C-reactive protein (CRP) levels of 23.40 mg/dl. The chest X-ray showed diffuse changes of bronchitis. His further laboratory investigations are mentioned in Table 1. His clinical condition improved over a period of time and there was a decrease in the count and CRP levels. His oral intake also improved gradually and thus was discharged after 3 days with the prescription of oral antibiotics. Following this, he had four bouts of respiratory tract infections with similar complaints and was treated on an outpatient basis. He underwent a 32 slice computed tomography (CT) scan with IV contrast which demonstrated non-visualization of the right main pulmonary artery and smaller right lung. The 2D echocardiography confirmed the hypoplastic right pulmonary artery with a small left-to-right patent ductus arteriosus shunt. The aortopulmonary collaterals were noted supplying to the right lung in the absence of the right pulmonary artery. These findings were in accordance with the scimitar syndrome variant with preferential blood flow to the left lung causing mild pulmonary artery hypertension.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Scimitar syndrome – Uncommon variant with common presentation

Dave¹,

Parikh²,

Vasoya³

2020

IJCR

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“…3,4,11 The risk for postoperative obstruction increases with circular direct anastomoses, acute angulation of veins, the need for increased mobilization, and handling or clamping of the veins at the time of surgery. 1,2,5,6,8 Because of their long vertical course, scimitar veins are particularly prone to this complication, with a reported obstruction rate of 15.5% to 23.8% 3,10,13,14 and reintervention rate of 11% to 30%. 3,10,13,14 Results after repair of scimitar syndrome are also variable 3,7-9,12-14 and are usually dictated by age at presentation, with comparably worse outcomes in the infantile form.…”

Section: Commentmentioning

confidence: 99%

“…1,2,5,6,8 Because of their long vertical course, scimitar veins are particularly prone to this complication, with a reported obstruction rate of 15.5% to 23.8% 3,10,13,14 and reintervention rate of 11% to 30%. 3,10,13,14 Results after repair of scimitar syndrome are also variable 3,7-9,12-14 and are usually dictated by age at presentation, with comparably worse outcomes in the infantile form. We previously reported the technique of in situ pericardial baffle to repair an acutely inserted scimitar vein, 8 and its successful application has since been reported by Sun and colleagues 12 in 9 of their patients with a vertical form of scimitar vein.…”

Section: Commentmentioning

confidence: 99%

Early Outcomes for In Situ Pericardial Roll Repair for Distant Anomalous Pulmonary Venous Return

Najm

Ahmad

Salam

et al. 2021

The Annals of Thoracic Surgery

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“…Только комплексная лучевая диагностика с включением мультиспиральной компьютерной томографической ангиографии (МСКТ-ангиографии) позволяет диагностировать синдром «ятагана», определить все его компоненты, уточнить анатомические особенности и определить объем и тактику дальнейшего кардиохирургического вмешательства [7,8]. В мировой литературе в большинстве своем представлены единичные случаи наблюдения синдрома [1,[9][10][11][12][13][14][15].…”

Section: Partial Anomalous Drainage Of Pulmonary Venous To the Inferiunclassified

Partial anomalous drainage of pulmonary venous to the inferior vena cava (scimitar syndrome): the part of radiated methods of research in primary diagnostic and control of surgical treatment

Basek¹,

Бенкен²,

Гребенник³

et al. 2020

Transl. med. (Print)

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Background. “Scimitar” syndrome is a rare congenital malformation with a frequency of 2 per 100,000 newborns. The syndrome is manifested by complete abnormal drainage of the pulmonary veins of the right lung into the inferior vena cava. According to the literature, a typical symptom of scimitar syndrome can be detected on radiographs in no more than 1/3 of patients. Only complex radiation diagnostics with the inclusion of multispiral computed tomographic angiography allows to diagnose “scimitar” syndrome, determine all its components, clarify the anatomical features and determine the volume and tactics of further cardiac surgery. Objective of the present clinical case is to assess the role of radiation research methods in the primary diagnosis and control of surgical treatment of scimitar syndrome. Materials and methods. A 39-year-old patient with a history of open arterial duct ligation and complaints of fatigue, shortness of breath arising from household loads was examined. According to the results of the chest x-ray and MSCT angiography of the chest organs, “scimitar” syndrome was diagnosed, the patient underwent a two-stage cardiosurgical treatment with the assessment of the results by MSCT angiography. Results. According to the results of radiation studies, the patient revealed a rare congenital malformation — “scimitar” syndrome. Conclusion. MSCT angiography of the chest is a highly informative, minimally invasive diagnostic method for the scimitar syndrome. MSCT angiography of the chest allows you to visually and accurately visualize the type of abnormal drainage, the course and place of the confluence of the collector, the presence of anastomoses with the left heart, which directly affects the definition of tactics for surgical correction of the disease. MSCT angiography of the chest allows you to evaluate the results of surgical treatment.

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Scimitar Syndrome in Children and Adults: Natural History, Outcomes, and Risk Analysis

Cited by 55 publications

References 17 publications

Scimitar syndrome – Uncommon variant with common presentation

Scimitar syndrome – Uncommon variant with common presentation

Early Outcomes for In Situ Pericardial Roll Repair for Distant Anomalous Pulmonary Venous Return

Partial anomalous drainage of pulmonary venous to the inferior vena cava (scimitar syndrome): the part of radiated methods of research in primary diagnostic and control of surgical treatment

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