scimitar syndrome


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scimitar syndrome

a radiographic artifact caused by a congenital disorder in which the right lower pulmonary vein drains into the inferior vena cava. On a chest radiograph, the abnormal vessel configuration produces a scimitar-shaped shadow.
A rare vascular anomaly which is more common in females that may present in early childhood, in which some or all of the pulmonary veins drain into the inferior vena cava. The scimitar syndrome is often accompanied by hypoplasia of the right lung (RL), dextrocardia, and systemic arterial supply to the RL from descending aorta
Surgical anatomy RL and right pulmonary artery hypoplasia, dextrocardia, anomalous origin of pulmonary arteries from the aorta to RL, anomalous venous drainage of RL into the inferior vena cava and anomalous right diaphragm
Management Pneumonectomy or correction of cardiovascular ‘plumbing’ defects.

scimitar syndrome

A rare congenital malformation of the heart and lungs, marked by dextroposition of the heart, a malformed right lung, abnormal connections between the right pulmonary veins and the inferior vena cava, and abnormal pulmonary arterial connections to the right lung.
References in periodicals archive ?
Scimitar Syndrome (SS) is also known as pulmonary venolobar syndrome or hypogenetic lung syndrome which is a rare congenital condition characterised by partial anomalous pulmonary venous connection of right lung to inferior vena cava, dextroposed heart, right lung hypoplasia.
Based on the above imaging tests and FOB findings, the diagnosis of scimitar syndrome with unilobar right lung was made.
9) The scimitar syndrome belongs to the group of venolobar syndromes associated with pulmonary sequestration and congenital heart defects.
The scimitar syndrome confirmed by 320-slice computerized tomography.
Scimitar syndrome is a very rare pathology which occurs with a rate of 2/100 000 and which presents in the infancy, childhood or adulthood with a female/male ratio of 2/1.
A novel approach to the surgical correction of scimitar syndrome.
Congenital heart defects that have been associated with UAPA are the followings; tetralogy of Fallot, ventricular septal defect (VSD), right aortic arch, truncus arteriosus, patent ductus arteriosus (PDA), CoA, subvalvular aortic stenosis, transposition of the great arteries and scimitar syndrome (2, 3, 5).
This case demonstrates how comprehensive, non-invasive imaging has replaced invasive techniques such as cardiac catheterisation and pulmonary angiography in the diagnosis of scimitar syndrome.
That patient is on mechanical ventilation with a previous failed extubation, severe pulmonary hypertension (PSP 52mmHG), and a diagnosis of scimitar syndrome by echocardiography.
Scimitar syndrome is a rare disorder that is associated with a range of congenital cardiac and pulmonary anomalies.
Thorax computed tomography revealed a connection between the inferior vena cava (IVC) and the right inferior pulmonary vein subdiaphragmatically, which was commented as the Scimitar syndrome (Fig.