An unusual variant of scimitar syndrome predisposing to recurrent pneumonia

Jackson, Niki; Nokes, Brandon; Sakata, Keiko; Cummings, Kristopher W.; Vaszar, Laszlo T.

doi:10.1016/j.rmcr.2019.01.010

Cited by 2 publications

(3 citation statements)

References 8 publications

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“…Recurrent pneumonia with scimitar syndrome may result from external airway compression by the collector of the pulmonary veins. Other mechanisms suggest impaired lymphatic drainage and insufficient local activity of lymphocytes and macrophages in the affected lung area [7]. Our patient did not actually have symptoms characte ristic of scimitar syndrome because there were no signs of heart failure, the girl did not have respiratory disease.…”

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confidence: 62%

Синдром Ятагана: Подходы К Диагностике И Ведению

Avramenko¹,

Kosmynina²

2021

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Âèïàäîê ³ç ïðàêòèêè / Case ReportDespite the continuous improvement of the methods of prenatal diagnosis of fetal malformations, the limited possibilities of intrauterine verification of vascular anoma lies of the lungs still take place. One of the most rare and difficult to diagnose forms of combination of vascular anomalies is partial abnormal pulmonary vein drainage, which is sometimes a component of scimitar syndrome (ICD-10: Q26.8) [1].Scimitar syndrome (hypogenetic pulmonary syndrome, venolobar syndrome or "yataghan" syndrome) belongs to a group of venolobar syndromes associated with lung sequestration and congenital heart disease. This is a congenital anomaly characterized by the fact that one or more pul-monary veins drain not into the left atrium, as normal, but directly into the inferior vena cava above or below the diaphragm. Scimitar syndrome accounts 4 % of abnormal pulmonary vein drainage. This syndrome is associated with changes in the arterial supply of the lungs, the right lung is hypoplasic, as well as the hypoplasic right bronchus and the right pulmonary artery. As far as the heart is concerned, in addition to dextroposition of the heart with displacement of the mediastinum, the most frequent changes (1/4 cases) are the absence of the pericardium, and other defects of development, such as defect of the atrial (60-70 %) or interventricular septum, coarctation of the aorta and tetralogy of Fallot (25 %).

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confidence: 62%

Синдром Ятагана: Подходы К Диагностике И Ведению

Avramenko¹,

Kosmynina²

2021

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“…The majority of older children who are diagnosed after the age of one remain asymptomatic, and adults are also diagnosed when characteristic chest radiographic findings are collected incidentally [16]. Recurrent pneumonia might be another problem that arise from extrinsic compression of an airway from the scimitar vein, although this may not be a unifying mechanism [17]. In our case, the patient was suffering from dyspnea and wheezing since her childhood and initially was diagnosed with allergic rhinitis and asthma, therefore, scimitar syndrome was diagnosed incidentally on chest radiograph, and later, by thorax CT angiogram.…”

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confidence: 99%

“…It is further associated with hypoplasia of the right lung, congenital heart malformation, an anomalous systemic arterial supply to the lung. Most often, the anomalous pulmonary vein drains into the inferior vena cava (IVC), but there are also case reports of the hepatic vein drainage into the right atrium and the left atrium [2]. Although the exact cause or any genetic mutation have not been identified, females are more frequently affected than males.…”

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confidence: 99%

A Rare Case Presented with Asthma Symptoms: Scimitar Syndrome

Tuğlu¹,

Öztürk²,

Ergün³

et al. 2019

AJMCR

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Background: Scimitar syndrome is a rare constellation of congenital conditions pertaining to partial anomalous pulmonary venous return to the inferior vena cava, which may associate with variable right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. Depending on the severity of the defect, it can present as early as in neonatal period or occasionally later in life. In many cases, there are also other associated cardiac anomalies. Case characteristics: 23-year-old woman with recurrent episodes of breathlessness and wheezing symptoms since her childhood. Computed tomography angiogram revealed right pulmonary veins draining into inferior vena cava through a single vein with a single ostium diagnosed as Scimitar syndrome. Outcome: Successfully managed with surgical correction. Message: Scimitar syndrome should be considered in adulthood with asthma symptoms if it is not under control.

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An unusual variant of scimitar syndrome predisposing to recurrent pneumonia

Cited by 2 publications

References 8 publications

Синдром Ятагана: Подходы К Диагностике И Ведению

Синдром Ятагана: Подходы К Диагностике И Ведению

A Rare Case Presented with Asthma Symptoms: Scimitar Syndrome

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