What Is Scimitar Syndrome?

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  • Written By: Mary McMahon
  • Edited By: O. Wallace
  • Last Modified Date: 06 April 2014
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Scimitar syndrome is a congenital heart defect which is characterized by an unusual arrangement of the pulmonary veins. In patients with this condition, one or more of the pulmonary veins from the right lung drains into the side of the heart which normally collects deoxygenated blood from the body so that it can be pushed into the lungs. This causes the heart to work harder than normal, and can lead to enlargement of the heart and other medical issues.

The severity of scimitar syndrome varies considerably. Some people have it and are entirely unaware, or have it diagnosed but a doctor determines that the effects are so minor that no action needs to be taken. In other cases, the defect causes health problems or puts the heart in danger, in which case surgery may be recommended to address the problem. This condition is also known as partial pulmonary venolobar syndrome.

In a patient with pulmonary venolobar syndrome, the veins have a distinctive appearance when they are viewed on X-ray, resembling the Middle Eastern weapon known as a scimitar and explaining the term "scimitar syndrome." The patient's pulmonary veins may drain fully or partially into the wrong side of the heart, and sometimes the heart is located on the right side of the chest instead of the left side. The affected lung and pulmonary veins tend to be smaller than normal, another finding which will be visible on X-ray.


This rare heart defect is usually diagnosed when someone appears to be experiencing heart problems and a doctor orders a medical imaging study to get an idea of what is going on inside the heart. Signs of heart problems can include irregular heartbeats, weakness, dizziness, difficulty breathing, and chest pain. Using an X-ray, a doctor can identify the tell-tale "scimitar sign" which indicates the presence of this defect, and determine how severe it is on the basis of anatomical changes which can be seen in the chest.

Some hereditary factors do appear to be involved with scimitar syndrome. In families with a history of this birth defect, children are more likely to be born with it. In other cases, it appears spontaneously, with no clear familiar history of the condition. It is important to keep in mind that no known family history is not the same as no family history. Someone in the family may have scimitar syndrome, but have such a mild case that it was never identified.


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Post 6

My baby was diagnosed with scimitar syndrome at 10 weeks old. She had dextrocardia, hypoplastic right lung, hypoplastic right pulmonary veins, ASD.

We spent 4.5 months in the intensive care unit, having repeated cardiac catheterisation and dilatation of pulmonary veins which were stenosed and closure of the atrial septal defect. She eventually succumbed to the illness at six and a half months. She ended up with pulmonary hypertension and right heart failure.

We did not know of any family history of such a disease. In fact, I didn't knew such a sickness existed until I was told about hers.

Post 5

I am now 66 years old. I was born late in the war years so scimitar syndrome was unknown. I have only been diagnosed during the past couple of years in spite of having symptoms for many years.

However, I am now under the care of a Congenital Heart Defect specialist and I am awaiting a decision on what can be done to improve my quality of life. I am not aware of anyone in my family with the syndrome.

Post 3

My son has Scimitar syndrome. It was diagnosed when he was 27 days of age. He was hospitalized for two and a half months. He was submitted to coil embolization of ASAS (Anomalous Systemic Arterial Supply).

He is now 10 months of age and he is ok. He is a small baby, but has lots of energy. He is still under surveillance to evaluate the need for further intervention.

Post 2

Wow. Thank you for that valuable piece of info, my granddaughter passed away six years ago of global enlarging of the heart, congenital heart failure, lower left ventricular dysfunction, and one other thing wrong with her heart. she was 18 months old. my oldest daughter has heart problems and I have a cousin that died at the young age of two years old. that sheds some light on things for me greatly.

Post 1

S.E Smith is sure a smart fellow! I appreciate him/her.

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