Scimitar syndrome
Scimitar syndrome is a rare congenital heart defect characterized by an abnormal arrangement of the pulmonary veins. The name "scimitar" refers to the distinctive shape of the abnormal vein on a chest radiograph, which resembles a curved Turkish sword known as a scimitar.
Symptoms and Diagnosis[edit | edit source]
The symptoms of Scimitar syndrome can vary greatly, ranging from no symptoms at all to severe respiratory distress. Common symptoms include shortness of breath, recurrent respiratory infections, and failure to thrive. The syndrome is often diagnosed in infancy, but in some cases, it may not be detected until adulthood.
Diagnosis of Scimitar syndrome typically involves imaging studies such as a chest radiograph or computed tomography (CT) scan. These tests can reveal the characteristic scimitar-shaped vein, as well as any associated heart defects.
Treatment[edit | edit source]
Treatment for Scimitar syndrome depends on the severity of the condition and the presence of any associated heart defects. In mild cases, no treatment may be necessary. In more severe cases, surgery may be required to correct the abnormal vein and any associated heart defects.
Prognosis[edit | edit source]
The prognosis for individuals with Scimitar syndrome varies depending on the severity of the condition and the presence of any associated heart defects. With appropriate treatment, many individuals with Scimitar syndrome can lead normal, healthy lives.
See Also[edit | edit source]
NIH genetic and rare disease info[edit source]
Scimitar syndrome is a rare disease.
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Contributors: Prab R. Tumpati, MD
