What Is Scimitar Syndrome?

Using an X-ray, doctors can evaluate telltale signs of scimitar syndrome, which is characterized by an unusual arrangement of the pulmonary veins.
Scimitar syndrome is a congenital heart defect which is characterized by an unusual arrangement of the pulmonary veins.
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  • Written By: Mary McMahon
  • Edited By: O. Wallace
  • Last Modified Date: 25 November 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.

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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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anon959567
Post 9

I was just recently diagnosed with scimitar syndrome a few days ago at 45 years old and have had problem after problem, like increased tiredness, weakening, shortness of breath, asthma-like symptoms, pneumonia and bronchial problems all my life. It got worse the older I got. It was blamed on allergies. I can’t even begin to tell you all the misdiagnoses I have had. Of course, the weaker and more short of breath I have become the less I do, thus the more I weigh at about 38 or so and about 180 pounds (I am 5 '7'). I could not pass a stress test and was sent to yet another heart doctor who did an EKG and told me all that was wrong with me was I needed to lose weight!

I finally landed in the ER. I couldn't breathe, my feet and hands were swelling up like balloons. I literally felt like I was drowning. The radiologist saw it on the X-ray and sent me to a great heart surgeon. I don't know yet, but it is looking like surgery soon, I am so happy to know what has been wrong all this time, and thankful something can be done about it!

anon947462
Post 8

My son has Scimitar Syndrome, but it was not diagnosed until he was 20 years old. All through his childhood, he suffered from one respiratory ailment after the other. Winters were horrible. He would have pneumonia several times. The doctors always attributed his problems to allergies. One even diagnosed him with "allergy induced asthma"and he was treated for many years.

It was only when he left home to go to college in the mountains that the real problems came to the surface. It took 17 doctors and four months of suffering before he was finally diagnosed. We would get phone calls in the middle of the night to tell us he had collapsed and was being taken to the hospital. He was three hours away from us and the fear of the unknown was unbearable. He was worsening by the day. We kept an overnight bag packed in each car in case a phone call came in.

It was through luck that he was diagnosed. An X-Ray technician saw a shadow that was odd looking and mentioned it to the doctor. By the grace of God, we had a diagnosis. He underwent 14 hours of corrective surgery to make the necessary repairs. His body had created a spiderweb like mass of vessels to compensate. His surgeon told us afterward that his case was so severe he should have never lived more than a few years.

My son was an extremely active child (and still is). He played sports year round. I shudder to think what could have happened. God was smiling on him. He is still here for a reason. I often wonder if we would have treated him differently had we known earlier. He is my hero and such an inspiration to those he meets. Every year we walk in the Heart Walk to raise money for research. Our donations go the support congenital heart defects research.

anon938169
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.

anon166804
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.

anon92861
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.

anon73800
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.

anon73775
Post 1

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

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