Scimitar vein syndrome: Report of nine new cases

Oakley, D.; Naik, D. R.; Vérel, D.; Rajan, Sujatha

doi:10.1016/0002-8703(84)90109-1

Cited by 25 publications

(19 citation statements)

References 4 publications

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“…Although a scimitar sign in a female patient with normal pulmonary venous connections may be called a pseudo-scimitar syndrome,9 it was an imprecise and unscrupulous nomenclature. In the literature, we found reports of so-called scimitar vein syndrome,13 scimitar variant,14,15 and incomplete scimitar syndrome.16 None of these 4 reports documented any systemic arterial supply or anomaly of the bronchial airway 13-16. Therefore, the patients in these reports were victims with the isolated scimitar vein anomaly.…”

Section: Discussionmentioning

confidence: 99%

“…The scimitar sign only refers to the curvilinear picture of the frontal chest film that mimics a scimitar (a curved Turkish sword). It should not be taken for granted that the presence of a scimitar sign implies the presence of a scimitar vein (anomalous pulmonary venous connection to the inferior vena cava), and vice versa , because these radiographic findings could be false positive8-11 or false negative12,13 in predicting presence of the scimitar vein (anomalous pulmonary venous connection to the inferior vena cava). Although the presence of a scimitar sign shall prompt us to search for the scimitar vein, neither the scimitar sign, nor the scimitar vein, nor the occurrence of both will fulfill the aforementioned diagnostic criteria for scimitar syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

Lee

2007

Yonsei Med J

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PurposeFour pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins.Materials and MethodsFrom January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies.ResultsClinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired.ConclusionThe clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

Lee

2007

Yonsei Med J

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“…It can be associated with hypoplastic right lung, pulmonary artery hypoplasia and dextrocardia, among others 1 2. There are complete and incomplete variations.…”

Section: Introductionmentioning

confidence: 99%

Anomalous pulmonary venous drainage: chest radiography and cardiac imaging

Athauda-arachchi

Mordi

Koch

et al. 2013

Postgraduate Medical Journal

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INTRODUCTIONScimitar syndrome (or pulmonary veno-lobar syndrome) is a rare congenital cardiac condition characterised by abnormal pulmonary venous return from the right lung directly into the inferior vena cava. It can be associated with hypoplastic right lung, pulmonary artery hypoplasia and dextrocardia, among others. 1 2 There are complete and incomplete variations. In the complete form, classically, the right lung is hypoplastic and is supplied by a systemic arterial branch from the aorta with hypoplasia of the right pulmonary artery, resulting in an abnormal right heart border on imaging.Most cases are diagnosed incidentally and are asymptomatic. Surgical treatment is indicated if there is a large left to right shunt (>50%) leading to pulmonary hypertension. Other indications also include lung sequestration, recurrent right-sided chest infections, or recurrent haemoptysis sometimes secondary to systemic arterial pressure supplying the vessels of the lung. 3 4We present a case of incomplete form of Scimitar syndrome picked up by review of chest radiography and include images from other complementary cardiac imaging modalities. CASE PRESENTATIONA 22-year-old woman, previously well, presented to our general medical unit with a cough and was investigated for a suspected chest infection. On admission, a chest radiograph was performed. This, somewhat surprisingly demonstrated radiographic features consistent with either a right-sided pleural effusion or volume loss ( figure 1A, thick arrow).To investigate further, contrast CT of the chest was performed. No pulmonary thromboembolism was demonstrated during the arterial phase of the study; however, a clear abnormality was seen in the venous phase. The presence of a rightsided anomalous (Scimitar) vein draining into the intrahepatic inferior vena cava (IVC) (figure 1B, arrow) was clearly visible, but no pulmonary sequestration was seen. In retrospect, this anomaly could be noted in the chest radiograph as well ( figure 1A, thin arrow). The CT confirmed that there was demonstrable left-sided pulmonary venous drainage, but total absence of any direct right-sided pulmonary venous drainage into the left atrium ( figure 1C, arrow).We undertook transthoracic and transoesophageal echocardiography to exclude associated cardiac anomalies and to reconfirm the absence of any direct right-sided pulmonary venous drainage into the left atrium. A right pulmonary vein (RPV) was draining into the intrahepatic portion of the IVC (figure 1D) was seen, and no right-sided pulmonary venous drainage into the left atrium was seen.

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“…Despite the varying spectrum of this syndrome, about half of the patients are asymptomatic or mildly symptomatic at the time of diagnosis [7]. Since the syndrome may be undetected in asymptomatic patients, the true incidence is difficult to determine [2,3].…”

Section: Introductionmentioning

confidence: 99%

Unique technique of surgery in an unusual variety of Scimitar syndrome: A Case Report

Nuebel

Januszewska

Loeff

et al. 2010

J Cardiothorac Surg

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Scimitar syndrome is a rare congenital anomaly characterized by total or partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We present a seven year old girl with a systolic murmur who was diagnosed as having a Scimitar syndrome with unusual drainage of the right pulmonary veins. The unique technique of surgery in this patient was appropriate to the unusual, previously not described anatomy.

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Scimitar vein syndrome: Report of nine new cases

Cited by 25 publications

References 4 publications

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

Anomalous pulmonary venous drainage: chest radiography and cardiac imaging

Unique technique of surgery in an unusual variety of Scimitar syndrome: A Case Report

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