Intraatrial conduit repair in Scimitar syndrome

A 26-year-old woman with partial anomalous pulmonary venous drainage into the right atrium (Scimitar syndrome) was successfully operated upon by incorporating an intra-atrial conduit. The single right pulmonary vein present was connected to the right atrial inferior vena cava junction. The atrial septum was extremely deviated to the left, and the left atrium was located entirely on the left of the spine. A small patent foramen ovale was found. The distance between the orifice of the right pulmonary vein and the enlarged atrial septal defect was too great to use an intracardiac patch, so a 14 mm woven Dacron graft, 6 cm long, was interposed between them. The patient is doing well 21 months following the operation.     

Scimitar syndrome in the newborn child and infant

We present 10 symptomatic patients with scimitar syndrome. Their age was below 1 year and four were newborn babies. In 5 patients there was secundum atrial septal defect and in one there was a patent ductus arteriosus. Sixty per cent of the patients had pulmonary sequestration. The right lung was moderately or severely hypoplasic in 8 cases and seven had frequent respiratory infections. The anomalous venous drainage produced a left-to right shunt at atrium level which in our series had a value of 2.87 +/- 0.5. Five patients had pulmonary hypertension either moderate or severe. The severity of this disease in early infancy is related to the pulmonary hypertension (40%), requiring a prompt surgical correction. Eight patients were operated upon, the drainage being connected to the left atrium. The six surviving patients are asymptomatic at 12 years of follow-up. Our series is the biggest published in our country and the second in the world literature in infancy.     

Scimitar syndrome in an adult - partial anomalous pulmonary venosus connection

Scimitar syndrome is a type of partial anomalous pulmonary venous connections, in which part or even the entire right lung is typically drained by right pulmonary veins that anomalously drain into the inferior vena cava. The affected lung is often hypoplastic. The severity of clinical signs and symptoms depend on the degree of a left-to-right shunt and the presence of pulmonary defects. We present a 60 year-old women who has been asymptomatic for a long time.     

Scimitar syndrome: imaging by magnetic resonance angiography and Doppler echocardiography

We report magnetic resonance angiographic demonstration of both an anomalous pulmonary venous drainage and an anomalous systemic arterial supply in a patient with scimitar syndrome. Contrast-enhanced magnetic resonance angiography provides an excellent non-invasive diagnostic tool for demonstrating this complex congenital lesion in detail. A two-dimensional and colour Doppler echocardiography was also performed to show the anomalous venous drainage and to analyse the anomalous flow velocity pattern.     

Scimitar syndrome--case report

Scimitar syndrome is a rare congenital anomaly. This syndrome is characterized by partial or complete anomalous pulmonary venous drainage of the right lung to the inferior vena cava. There is a characteristic abnormal radiographic shadow which descends along the right cardiac border (scimitar sign). We examined 71-year old woman with severe pulmonary hypertension due to a large shunt between pulmonary veins and right atrium. Other cause of pulmonary hypertension is atrial septum defect. Our patient required permanent pacemaker implantation for tachy-brady syndrome.     

A familial variant of the Scimitar syndrome with a meandering pulmonary vein

We report a rare variant of the Scimitar syndrome, in which the right lower pulmonary vein takes a meandering course before finally connecting in normal fashion to the left atrium. The pulmonary parenchymal segment drained by the tortuous vein is supplied by a systemic collateral artery, which was coiled via a catheter. We also closed the intracardiac defects by surgery. The elder brother of the patient had classical Scimitar syndrome.