scimitar syndrome


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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 ?
Keywords: Scimitar syndrome, Dextroposition of heart, Pulmonary hypoplasia.
Meandering pulmonary vein (MPV) is a rarely seen pulmonary vascular anomaly and confused with the scimitar syndrome or solitary pulmonary nodules.
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.
The most common variant of PAPVC is the defect located in sinus venosus malformation i.e, superior caval atrial septal defect coexists with PAPVC.2,3 Other variants include right pulmonary vein draining into right atrium,4 connection of right pulmonary vein to IVC (scimitar syndrome) and rarely right pulmonary vein connects to azygos vein or coronary sinus.5,6 Similarly, left pulmonary vein may connect to left brachiocephalic vein through an anomalous vertical vein.
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 partial anomalous venous return of the pulmonary vein to the inferior vena cava (IVC) rather than directly to the left atrium.
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.
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).
(8) The term 'scimitar syndrome' first appears in the 1960 article by Neill et al., referring to the combination of anomalous venous drainage of all or most of the right lung to the right atrium or IVC together with hypoplasia of the right lung and associated with systemic arterial collaterals.
6) confirmed the diagnosis of scimitar syndrome, which is characterised by partially anomalous pulmonary venous drainage, pulmonary lobar hypogenesis and cardiac dextroposition.
That patient is on mechanical ventilation with a previous failed extubation, severe pulmonary hypertension (PSP 52mmHG), and a diagnosis of scimitar syndrome by echocardiography.