Scimitar syndrome is also called as pulmonary venolobar syndrome. It is a rare condition with congenital etiology. This congenital cardiac disorder has an anomalous venous return from the right pulmonary, which is more often partial.
In a normal pulmonary venous circulation the oxygenated blood is exchanged from the capillaries to the left side of the heart.
In the Scimitar syndrome, the anomalous vein shunts the blood to the inferior vena cava, which in an x-ray resembles a scimitar shape.
Scimitar syndrome is associated with hypoplasia of the right lung, hypoplasia of the pulmonary artery and pulmonary sequestration (or a cystic lung lesion). Often it is associated with dextroposition of the heart as well.
The common symptoms associated with scimitar syndrome are –
1. Shortness of breath
2. Rapid breathing
3. Respiratory distress
4. Cyanosis (Bluish hue)
5. Irregular heartbeat
6. Weakness
7. Dizziness and chest pain
8. Retraction of the muscles of the rib cage.
This hereditary condition is confirmed on CT angiography, MR angiography, and trans-thoracic or trans-esophageal echocardiography. Scimitar syndrome can lead to pulmonary hypertention, and presence of left to right shunting (the anomalous vein drains into the Inferior vena cava in most cases, right atrium or the portal vein systemic) needing surgical correction.
