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Scimitar syndrome is a congenital heart defect where one or more pulmonary veins from the right lung drain into the wrong side of the heart, causing the heart to work harder. It can vary in severity and is usually diagnosed through medical imaging. It may have hereditary factors.
Scimitar syndrome is a congenital heart defect characterized by an unusual arrangement of the pulmonary veins. In patients with this condition, one or more pulmonary veins from the right lung drain into the side of the heart that normally collects deoxygenated blood from the body so it can be pushed into the lungs. This causes the heart to work harder than normal and can lead to heart enlargement and other medical problems.
The severity of scimitar syndrome varies considerably. Some people have it and are completely unaware of it, or have it diagnosed, but a doctor determines that the effects are so mild that no action is needed. In other cases, the defect causes health problems or endangers the heart, in which case surgery to address the problem may be recommended. This condition is also known as partial pulmonary venolobar syndrome.
In a patient with pulmonary venolobar syndrome, the veins have a distinctive appearance when viewed on X-rays, resembling the Middle Eastern weapon known as the scimitar and explaining the term “scimitar syndrome.” The patient’s pulmonary veins may drain completely or partially into the wrong side of the heart, and sometimes the heart is 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 that will be visible on X-rays.
This rare heart defect is usually diagnosed when someone appears to have heart problems and a doctor orders a medical imaging study to get an idea of what’s 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 telltale “scimitar mark” that indicates the presence of this defect and determine its severity based on the anatomical changes that can be seen in the chest.
Some hereditary factors appear to be involved in scimitar syndrome. In families with a history of this birth defect, babies are more likely to be born with it. In other cases, it appears spontaneously, with no clear family history of the condition. It is important to keep in mind that no known family history is not the same as no known family history. Someone in the family may have scimitar syndrome, but have a case so mild that it has never been identified.
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