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Heart Consult

Scimitar Syndrome

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. 
Scimitar sword'_0.jpg
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. 
scimitar syndrome
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. 


scimitar syndrome

i had xray 2 weeks ago and they sent for me to be re xrayed yesterday saying first xray may show a scimitar syndrome with over laying shadow although my latheral left to right xray was clear? i havnt had results of yesterdays xray yet but as i suffer from shortness of breath weezing chest and somtimes get strange feeling round my heart like its racing,iv also in the past had pain like iv pulled a muscle in my chest,do you think this sounds like scimitar syndrome?any advice welcome.

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I got that too...

I have it. Diagnosed at 33 years of age on a fluke. Had a diag procedure for IBS. While examining my digestive tract the tech noticed the abnormally which was later diag as Scimatar. 6 extensive diag procedures, no surgerys and 15 years later Im still here. Don't let the Cardiologist scare you into unnecessary surgery just to exam you like a disected frog in lab. Get a 2nd and 3rd option and live your life without fear.

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