Scimitar Syndrome

Submitted by webmaster on Tue, 10/23/2018 - 15:21

Scimitar Syndrome is a rare congenital heart disease which is characterized by an unusual arrangement of the pulmonary veins. Scimitar syndrome is also called as the pulmonary venolobar, Halasz’s syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, epibronchial right pulmonary artery syndrome and venacava bronchovascular syndrome. It occurs more commonly in females when compared to males. 

Normal pulmonary venous circulation carries oxygenated blood from the capillaries to the left side of the heart. But in case of the Scimitar Syndrome, an anomalous vein connects between the pulmonary venous circulation and systemic venous circulation which creates a left-to-right shunt. Scimitar syndrome is a rare congenital anomaly in which a part of the right pulmonary venous blood return to the inferior vena cava. When seen in an X-ray the anomalous vein resembles the shape of a scimitar and hence the name scimitar syndrome. 

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Symptoms of Scimitar syndrome

A few of the common symptoms of Scimitar Syndrome are:

  • Rapid breathing
  • Cyanosis 
  • Respiratory distress 
  • Shortness of breath
  • Rib cage muscle retraction


Though the causes for Scimitar syndrome are unknown, it is a birth defect and there are high chances of children to be born with in it, if it prevails in the family. Yes, you got it right. Scimitar syndrome has got much to do with the family history that is,it is associated with some of the hereditary factors.  

Tests and diagnosis

The severity of scimitar syndrome varies significantly. There are cases, wherein some people have it and are not aware of it until it is diagnosed whereas in other cases, the defect causes serious health problems. 


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The diagnosis for Scimitar is done for certain people ailing with heart problems like weakness, irregular heartbeat, dizziness, chest pain, etc. The doctor confirms the defect with the help of the medical imaging services which gives a clear cut idea to the doctor for what exactly is happening in the heart. If this defect is present, then the doctor can identify the scimitar sign with the help of an x-ray . Other diagnostic signs for scimitar syndrome includes pulmonary sequestration, unilateral absence of pulmonary artery, and right middle lobe atelectasis. 


Surgical correction should be considered in the presence of significant left to right shunting and pulmonary hypertension.   The treatment of scimitar syndrome involves the creation of an inter-atrial baffle in order to redirect the pulmonary venous return into the left atrium. Another option is to re-implant the anomalous vein into the left atrium directly. However, the decision on which treatment to choose solely depends on the doctor. It depends on various factors like the amount of blood flowing to the inferior vena cava, the extent of shunting of the left to right, etc. If at all you are having any of the symptoms of scimitar syndrome, then it is always advisable to seek able guidance from your doctor.