Scimitar syndrome with anomalous connection of left superior pulmonary vein to left innominate vein

Misra, Manoranjan; Sadiq, Adil; Manohar, Krishna; Neelakandhan, Kurur Sankaran

doi:10.1510/icvts.2005.111500

Cited by 11 publications

(5 citation statements)

References 4 publications

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“…Bilateral partial anomalous pulmonary venous connection is very rare with only a few cases being reported, the most common variant being the anomalous connection of the left superior pulmonary vein to the left innominate vein and right superior pulmonary vein to the superior vena cava-right atrium junction [28]. Awareness of such variants of bilateral partial anomalous pulmonary venous connection is required for recognition and early successful single-stage complete correction.…”

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et al. 2010

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Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome.

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et al. 2010

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“…The first anatomical observation of this peculiar anomalous venous connection was described by Cooper and Chassinat in 1836 but the term “scimitar” did not appear in their reports. This anomaly accounts for 3% to 5% of partial anomalous pulmonary venous connection 2 but the true incidence may be higher in “adult” forms because many patients remain asymptomatic. Available small retrospective series and review confirm that “infantile” forms of the syndrome have a higher incidence of symptoms, aortopulmonary collaterals, associated congenital heart defect, extracardiac anomalies, and pulmonary hypertension and have a worse prognosis compared with patients diagnosed later 3 .…”

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confidence: 99%

Adults forms of scimitar syndrome

et al. 2020

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Scimitar syndrome is a rare malformation defined as the partial or total anomalous pulmonary venous return of the right lung veins to the inferior vena cava just above or below the diaphragm. Severe forms of the disease are diagnosed in infancy and childhood. However, because of the mild form of the syndrome in adult patients, they remain asymptomatic and few cases are reported in the literature. We report two adults cases of this syndrome with one presenting with chest discomfort.

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“…[5] Scimitar syndrome is a rare complex variant of PAPVR (3-5%), occurring in 1-3/100000 live births and consists of PAPVR, hypoplasia of the right lung and pulmonary artery, dextroposition of the heart, with right pulmonary arterial supply from the systemic circulation. [678] Mostly, the pulmonary vein from the right lower lobe and occasionally the right middle lobe forms an abnormal ‘Scimitar’ vein that drains into the upper IVC below the level of the diaphragm. [7] Consequently, a left to right shunt is established, with risk of right heart failure due to right ventricular volume overload.…”

Section: Discussionmentioning

confidence: 99%