Eponym

Abstract

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.

Incidental detection of a curved radiopacity on the chest X-ray

“…[5] The word “Scimitar” is used to describe this syndrome as the anomalous pulmonary vein of right lung appears as a curved radiopacity along the right cardiac border on chest radiograph, resembling a curved Turkish sword called scimitar. [6] Surgical treatment is only indicated in scimitar syndrome if left to right shunt is significant or severe symptoms and right ventricular dilatation and concomitant cardiac lesions are seen. [7] The type of surgical management to be used is controversial and includes reimplantation of anomalous pulmonary vein into the left atrium, division of abnormal arterial supply of the lung, or partial/complete resection of the right lung.…”

Transcatheter intervention in a child with scimitar syndrome

“…Recently, multiple reports have shown transcatheter intervention, including embolisation of the APC and closure of the cardiac defects, may improve symptoms, decrease pulmonary arterial pressure,11,12 and postpone or even eliminate the need for surgical correction 7,8,13. Instead of a surgical approach, patients with scimitar syndrome who have significant left-to-right shunt due to APC and other cardiac defects are largely suitable for transcatheter intervention.…”

“…Pneuomonectomy or lobectomy is performed cautiously in patients with persistent haemoptysis, thrombosed intra-atrial baffle, obvious hypoplasia of the right lung,14 or right pulmonary vein obstruction at risk of scoliosis post operation2, and respiratory insufficiency post pneuomonectomy 10,12,15…”

Multimodality Imaging in an Adult Patient with Scimitar Syndrome

“…The “Scimitar syndrome” is a rare congenital anomaly characterized by combination of partial or complete pulmonary venous return from the right lung to the inferior vena cava either above or below the diaphragm together with hypoplasia of the right lung and sometimes systemic collateral vessels to the right lung (1,2,3). Descending vein toward the diaphragm on the right of the heart produces a curvilinear avascular shadow, resembling a curved Turkish sword named “scimitar” on the chest radiograph, and therefore the syndrome derives its name from this appearance (2).…”

“…Descending vein toward the diaphragm on the right of the heart produces a curvilinear avascular shadow, resembling a curved Turkish sword named “scimitar” on the chest radiograph, and therefore the syndrome derives its name from this appearance (2). The insults causing this malformation in early embryogenesis are unknown.…”