Last reviewed by mvhs13 on August 13th, 2018.
Scimitar Syndrome is a rare congenital heart defect. It is also referred to as partial pulmonary venolobar syndrome. Scimitar syndrome gets its name from the distinctive appearance they show when they are viewed on x-rays. On the x-rays they resemble a Middle Eastern weapon called the scimitar, which is a curved sword. It generally affects a person in their early childhood and may even be present at birth. Scimitar syndrome occurs in one to three in every one hundred thousand births. This number could be higher if the person shows no signs of this syndrome for most of their lives. Females are more likely to develop scimitar syndrome than males.
What happens with Scimitar syndrome?
With scimitar syndrome one or more pulmonary veins from the right lungs drains into the side of the heart that normally collects blood from your body that is deoxygenated so it can be pushed into your lungs. Your pulmonary veins are the large blood vessels that receive the oxygenated blood from your lungs. In scimitar syndrome the drainage into the wrong side of your heart can be partially or fully drainage. When this happens it will cause your heart to work harder than it normally does, which can lead to the enlargement of your heart along with other medical issues.
How severe scimitar syndrome will be varies from one person to another. In some they may be entirely unaware that they have it or when it is diagnosed the physician determines that the effects are so minor that no treatment is needed. There are other cases that can put your heart in dance or causes health problems. If this is the case then surgery is the recommended treatment.
Symptoms and Characteristics
Two of the characteristics of scimitar syndrome are the unusual arrangement of their pulmonary veins and the affected pulmonary veins and lung tend to be smaller than normal. In some cases a person may not have any symptoms but in others they may experience:
- Rapid breathing
- Shortness of breath
- Cyanosis, which is having a bluish appearance to your mucous membranes and skin due to lack of oxygen in your blood
- Respiratory distress
- Retraction of your rib cage muscle
If a child is born with scimitar syndrome symptoms may appear shortly after they are born or in early childhood. Some of these symptoms can include:
- Easily becoming fatigued
- Struggling for breath
- May show signs of bluing in their extremities, which indicates their body is not getting enough oxygen.
The exact cause of a person having scimitar syndrome is not known but in some cases it appears that it is hereditary, especially if families have a history of this type of birth defect. If it is genetic it will happen during fetal development. Other times it appears spontaneously. There are even some whose heart is located on the right side of their chest instead of on their left side.
Scimitar syndrome is usually diagnosed when a person appears to be experiencing heart problems and is having a medical imaging test done to see what is happening inside their heart. When looking at the chest x-ray the physician can identify the “scimitar sign” that lets them know about this defect. The chest x-ray can also help the physician determine how severe it is based on the anatomical changes seen in their chest.
If the person is not showing any symptoms treatment is normally not needed but they do need to under constant observation to see if they are showing any signs of deterioration. If they are showing symptoms prompt treatment is needed early to avoid any serious complications. The most common treatment is with surgery. One possible issue with what treatment is used is if it is a severe case then you may have other abnormalities, which could create complications if you have surgery. These abnormalities may also cause you to wait for surgery to correct the problems with scimitar syndrome. Before deciding on the treatment and when it is going to happen you will need to have a careful evaluation.
When surgery is done the cardiologist surgeon will create a connection between the right and left sides of your heart to allow the oxygenated blood to drain properly. Another surgical option is to reposition the blood vessel that is abnormal. Which surgery would be best would depend on your anatomy, the specifics of your case, and the experience of the cardiologist surgeon.
After the surgery some lead highly active lives and have no further complications. There are others who may need to have additional surgery along with follow-ups because of some complications that were related to other medical conditions or because of the syndrome. What the actual prognosis will be depends on how severe the scimitar syndrome was.
If a child is diagnosed with scimitar syndrome at birth or during their first year they are more likely to have a poor life expectancy but with proper treatment they may be able to live a long life. Unfortunately most have a short life span. If scimitar syndrome develops when they are an adult the mortality rate is lower.