Scimitar syndrome: report of a case and its surgical management

Rajaii-Khorasani, Ahmad; Kahrom, Mahdi; Mottaghi, Hassan; Kahrom, Hadi

doi:10.4103/0256-4947.51820

Cited by 8 publications

(10 citation statements)

References 6 publications

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“…Puig-Massana and Revuelta [34] described use of the free wall of the right atrium to create a tunnel from the scimitar vein to the left atrium across an ASD. There have been two case reports utilizing a 14-mm Dacron graft [44] and a 20-mm Dacron graft [2] interposed between the orifice of the anomalous pulmonary vein and an enlarged ASD as an intra-atrial conduit [35].…”

Section: Treatment Modalities and Prognosismentioning

confidence: 99%

“…Surgical options in such cases include redirecting the venous drainage to the left atria, ligation/embolization of vascular supply to the sequestered lobe, and pneumonectomy [39]. The first report of surgical therapy for scimitar syndrome was in 1950 by Drake and Lynch, who performed a right lower lobectomy [6,35]. Kirklin et al [23] in 1956 tried the first total correction without cardiopulmonary bypass, to avoid lung resection and complications associated with simple ligation of the anomalous pulmonary vein.…”

Section: Treatment Modalities and Prognosismentioning

confidence: 99%

“…These abnormalities include atrial septal defect, ventricular septal defect, coarctation of the aorta, abnormalities of the aortic arch, and abnormal relationship of the pulmonary arteries and bronchi [20] (Table 2). Usually, the atrial septum is intact; however, atrial septal defect (ASD) is the most frequentrelated congenital heart abnormality, with ostium secundum being the most common [35]. Hemodynamically, there is an acyanotic left to right shunt; the anomalous pulmonary vein drains blood from the right lung into the IVC resulting in an increased risk of developing right ventricular failure due to long-standing right ventricular volume overload [19].…”

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confidence: 99%

“…Scimitar syndrome constitutes 0.5% to 1% of all congenital heart diseases. This rare anomaly has an incidence of approximately one to three per 100,000 live births; however, the true incidence may be higher because many patients remain asymptomatic [35].…”

mentioning

confidence: 99%

“…The appearance was termed the "scimitar sign" because the aberrant venous return resembles a scimitar, which is a short, curved Turkish sword (sometimes the word "shimshir" is also used which is a Persian sword; Fig. 1) [20,24,35]. Typical scimitar sign may not be seen in every patient in infancy.…”

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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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Section: Treatment Modalities and Prognosismentioning

confidence: 99%

Section: Treatment Modalities and Prognosismentioning

confidence: 99%

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confidence: 99%

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et al. 2010

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Scimitar syndrome and pregnancy, complicated with severe preeclampsia

Yüksel

İmamoğlu

Çakmak

et al. 2014

Arch Gynecol Obstet

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Scimitar syndrome (pulmonary venolobar syndrome) is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. This is the report of a case of a 29-year-old woman at 31 weeks of gestation of pregnancy who was previously diagnosed with scimitar syndrome. MR angiography and PET-CT results which were obtained before pregnancy demonstrated vascular malformation in the inferior part of the right lung. No specific treatment was planned throughout the pregnancy due to the absence of any symptoms. The patient's first physical examination was unremarkable except mild hypertension. In her follow-up, severe preeclampsia was developed and the patient had undergone a cesarean section of a live birth at 34 weeks and 2 days of gestation. This is the first case of scimitar syndrome with pregnancy in which the cardiac status of the patient deteriorated coincidentally due to the development of another manifestation such as severe preeclampsia besides the syndrome itself.

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Right-sided scimitar syndrome in a patient with a single aortic trunk and coronary-cameral venous fistula

Çilingiroğlu

Akkuş

2013

Revista Portuguesa de Cardiologia

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Scimitar syndrome (SS) 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, persistent left superior vena cava, and dextroposition of the heart. We report a rare variant of SS in a 44-year-old man together with a single aortic trunk, as well as a coronary-cameral venous fistula.

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Scimitar syndrome: report of a case and its surgical management

Cited by 8 publications

References 6 publications

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Scimitar syndrome and pregnancy, complicated with severe preeclampsia

Right-sided scimitar syndrome in a patient with a single aortic trunk and coronary-cameral venous fistula

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