Congenitally palliated scimitar syndrome

Cantinotti, Massimiliano; Giordano, Raffaele; Spadoni, Isabella

doi:10.1017/s1047951114001826

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Additionally, repair of the anomalous pulmonary venous return in symptomatic patients with moderate to severe left to right shunting and associated right ventricular strain and pulmonary hypertension is required (15). Interestingly, there is a reported case of congenitally stenotic scimitar vessel in which the scimitar vessel was stenotic at the junction of the IVC allowing for the majority of the oxygenated blood from the lung to be drained into the left atrium and hence not requiring repair of the anomalous pulmonary venous return (16). This case exemplifies the need for detailed and objective assessment of the degree of shunting, in order to better guide therapy.…”

Section: Discussionmentioning

confidence: 99%

Not all right-sided hearts are the same—the importance of identifying the correct diagnosis

Siddiqui

Rubio

Patel

et al. 2016

Annals of Translational Medicine

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Scimitar syndrome is characterized by an anomalous venous return with the characteristic chest roentgenogram (CxR) appearance of the anomalous vein draining into the inferior vena cava (IVC). This appears as a curvilinear opacity paralleling the right border of the heart resembling a curved sword or Scimitar. A 27-year-old white woman with a reported history of dextrocardia was admitted after a drug overdose. Examination demonstrated an obtunded woman with tachycardia and right sided heart sounds. Her CxR revealed a right sided heart image with two curvilinear opacities in the retrocardiac area. Chest computed tomography (CT) demonstrated that these opacities join to represent an anomalous vein draining into IVC. Furthermore, an anomalous systemic artery arising from the abdominal aorta was seen to supply the right lower lobe. The patient was eventually diagnosed with Scimitar syndrome. This syndrome affects 1-3 in 100,000 live births while nearly half of the patients remain asymptomatic with some initially being misdiagnosed as dextrocardia, such as in our case. Correctly diagnosing these patients is of paramount importance as some can develop severe pulmonary hypertension and right ventricular failure. In turn, close ongoing echocardiographic monitoring can help identify those that may benefit from surgical interventions to prevent them from developing these complications.

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Section: Discussionmentioning

confidence: 99%

Not all right-sided hearts are the same—the importance of identifying the correct diagnosis

Siddiqui

Rubio

Patel

et al. 2016

Annals of Translational Medicine

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show abstract

“…A scimitar vein draining to the left atrium is a rare form of variant with only a few cases described in the literature 12 14 , 15 Left-sided variants of the scimitar syndrome have also been described with hypoplasia of the left lung and pulmonary artery, an anomalous systemic blood supply from the abdominal aorta to the lower segments of the left lung, and an anomalous left pulmonary venous drainage to the inferior caval vein or azygos vein 9 16 Bilateral partial anomalous pulmonary venous drainage is also encountered, the most common type being an anomalous drainage of the left superior pulmonary vein to the left innominate vein and right superior pulmonary vein to the superior caval vein 17 .…”

Section: Discussionmentioning

confidence: 99%