Scimitar syndrome
Scimitar Syndrome
Scimitar Syndrome (pronounced: /ˈsɪmɪtər/), also known as Pulmonary Venolobar Syndrome, is a rare congenital heart defect characterized by anomalous pulmonary venous return.
Etymology
The term "Scimitar" is derived from the Turkish word "scimitar", which refers to a curved sword. The syndrome is named so because the anomalous vein, seen on a chest X-ray, is shaped like a scimitar sword.
Definition
Scimitar Syndrome is a congenital disorder that involves several abnormalities in the structure of the heart and lungs. The most common feature is the partial or total anomalous pulmonary venous return (PAPVR or TAPVR) of the right lung to the inferior vena cava, which is often associated with a right lung hypoplasia and dextroposition of the heart.
Symptoms
Symptoms of Scimitar Syndrome can vary greatly, but often include respiratory distress, recurrent pneumonia, and failure to thrive. Some individuals may remain asymptomatic.
Diagnosis
Diagnosis of Scimitar Syndrome is typically made through imaging studies such as a chest X-ray, CT scan, or MRI. The characteristic "scimitar sign" can often be seen on a chest X-ray.
Treatment
Treatment for Scimitar Syndrome is typically surgical and aims to correct the anomalous venous return. The specific surgical procedure will depend on the individual's specific abnormalities.
Prognosis
The prognosis for individuals with Scimitar Syndrome can vary greatly depending on the severity of the condition and the presence of other health issues. With appropriate treatment, many individuals can lead normal lives.
Related Terms
- Congenital disorder
- Pulmonary venous return
- Respiratory distress
- Pneumonia
- Failure to thrive
- X-ray
- CT scan
- MRI
External links
- Medical encyclopedia article on Scimitar syndrome
- Wikipedia's article - Scimitar syndrome
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