Natural History and Clinical Outcome of “Uncorrected” Scimitar Syndrome Patients: a Multicenter Study of the Italian Society of Pediatric Cardiology

Vida, Vladimiro L.; Padrini, Maddalena; Boccuzzo, Giovanna; Agnoletti, Gabriella; Bondanza, Sara; Butera, Gianfranco; Chiappa, Enrico; Marasini, Maurizio; Pilati, Mara; Pongiglione, Giacomo; Prandstraller, Daniela; Russo, Maria Giovanna; Castaldi, Biagio; Santoro, Giuseppe; Spadoni, Isabella; Stellin, Giovanni; Milanesi, Ornella

doi:10.1016/j.rec.2013.03.008

Cited by 23 publications

(80 citation statements)

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“…Another multicenter study exploring the natural history of patients with a scimitar vein in 44 individuals found that the course is often benign when no other congenital cardiac abnormalities are present. However, when it was associated with other cardiac defects or pulmonary hypertension, there was an increased risk of heart failure and mortality …”

Section: Discussionmentioning

confidence: 99%

“…However, when it was associated with other cardiac defects or pulmonary hypertension, there was an increased risk of heart failure and mortality. 8 The chest X-ray may provide the first clue to scimitar syndrome, but the initial diagnosis is usually made by echocardiography 9 and confirmed by other imaging modalities like MRI or CT. The diagnosis can also be made at cardiac catheterization, by pulmonary arterial angiography or retrograde contrast injection of the anomalous vein.…”

Section: A S Ementioning

confidence: 99%

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Multimodality imaging of scimitar syndrome in adults: A report of four cases

et al. 2018

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Partial anomalous pulmonary venous return (PAPVR) comprises a group of congenital cardiovascular anomalies associated with pulmonary venous flow directly or indirectly into the right atrium. Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins connected anomalously to the inferior vena cava. Surgery is the definitive treatment for scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left-to-right shunts, and enjoy a normal life expectancy without surgery. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: A S Ementioning

confidence: 99%

Multimodality imaging of scimitar syndrome in adults: A report of four cases

et al. 2018

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“…Similarly, Vida et al 15 performed a retrospective study on 44 patients with scimitar syndrome. None of those patients underwent correction of the anomalous scimitar vein; instead, 18% underwent correction of the associated CHD and 34% had embolisation of the anomalous systemic arterial supply if present.…”

Section: Discussionmentioning

confidence: 99%

Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman

2017

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Scimitar syndrome is the constellation of malformations including an abnormal venous drainage of the right lung into the inferior vena cava, associated with the right lung and systemic supply to the right lung. The anomalous vein looks like the curved, Turkish sword (scimitar), hence the name.The adult form of scimitar syndrome is rare, and it is usually an incidental diagnosis based on the characteristic finding on radiological imaging since the patients are usually asymptomatic or with minimal symptoms.Our patient presented with a rare presentation of scimitar syndrome, which is tachyarrhythmia (sinus tachycardia, with episodes of supraventricular tachycardia). The diagnosis of scimitar syndrome was made based on the typical radiological finding of the anomalous venous drainage on CT angiography. Our patient does not have the full spectrum of the scimitar syndrome; therefore, she did not suffer from the usual complication (pulmonary hypertension). She was treated with ablation without surgical intervention.

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“…Bronkoskopide sağ ara bronş yokluğu dikkatimizi çekmişti ve MR anjiografi tanımızı kesinleştirmişti. Scimitar sendromunda diğer kardiyak defektler ve pulmoner hipertansiyonun eşlik ettiği durumlarda, konjestif kalp yetmezliği ve mortalite riski artmaktadır (18). Cerrahi için endikasyonlar; kalp yetmezliği ve pulmoner hipertansiyona yol açan %50'nin üzerinde sol-sağ şant, pulmoner sekestrasyon ve/veya rekürren sağ akciğer kaynaklı enfeksiyonlar olarak kabul edilir (18).…”

Section: şEkil 4: Toraks Manyetik Rezonans (Mr) Anjiografide Sağ Pulunclassified

Scimitar Syndrome: A Case Report

Aksoy¹,

Tokyay²,

Akyıl³

et al. 2015

Respir Case Rep

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Scimitar sendromu veya pulmoner venolober sendrom, nadir görülen bir konjenital venöz dönüş anomalisidir. Kırk yedi yaşında kadın hasta, göğüs ağrısı ve eforla gelişen nefes darlığı şikâyeti ile başvurdu. Fizik muayenesi ve laboratuvar bulguları normaldi. Postero-anterior akciğer grafisinde sağ akciğer alt alanda sağ kalp kenarı boyunca nonhomojen opasite artışı, sağ akciğerde volüm kaybı, kalp gölgesinin sağa doğru yer değiştirdiği izlendi. Ekokardiyografide sistolik pulmoner arter basıncı 55 mmHg ölçüldü. Fiberoptik bronkoskopide sağ bronşial sistemde ara bronşun olmadığı görüldü. Manyetik rezonans anjiografide, Scimitar sendromu ile uyumlu olarak sağ pulmoner venlerin subdiafragmatik mesafede inferior vena kavaya drene olduğu izlendi. Anormal pulmoner venöz dönüş, sağ akciğer hipoplazisi, ara bronş anomalisi ve pulmoner hipertansiyon ile Scimitar sendromu tanısı konulan olgumuz literatürler eşliğin-de sunuldu.Anahtar Sözcükler: Pulmoner hipertansiyon, pulmoner ven, scimitar sendromu.Scimitar sendromu olarak bilinen venopulmoner sendrom, sağ pulmoner venin, inferior vena kava veya sağ atriuma açılması ile karakterize nadir görülen konjenital bir pulmoner venöz dönüş anoScimitar syndrome or pulmonary venolabar syndrome is a rare congenital anomalous pulmonary venous return. A 47-year-old woman presented at our clinic with chest pain and shortness of breath during exertion. The physical examination and laboratory findings were normal. The chest radiograph demonstrated non-homogeneous increased opacity along the right cardiac border, and volume loss of the right lung with cardiac silhouette displacement, to the right. Echocardiography pulmonary artery systolic pressure was measured as 55 mmHg. Fiberoptic bronchoscopy revealed the intermediate bronchus was absent. On magnetic resonance angiography, it was observed that the right pulmonary veins drained into the inferior vena cava at a subdiaphragmatic level, consistent with Scimitar Syndrome. The case, diagnosed as Scimitar syndrome, is presented in the light of the literature.

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Natural History and Clinical Outcome of “Uncorrected” Scimitar Syndrome Patients: a Multicenter Study of the Italian Society of Pediatric Cardiology

Cited by 23 publications

References 24 publications

Multimodality imaging of scimitar syndrome in adults: A report of four cases

Multimodality imaging of scimitar syndrome in adults: A report of four cases

Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman

Scimitar Syndrome: A Case Report

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