Scimitar syndrome

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Scimitar syndrome
Synonyms	Pulmonary venolobar syndrome, Hypogenetic lung syndrome
Pronounce
Specialty	Cardiology, Pulmonology
Symptoms	Shortness of breath, Cyanosis, Recurrent respiratory infections
Complications	Pulmonary hypertension, Heart failure
Onset	Congenital
Duration	Chronic
Types
Causes	Congenital heart defect
Risks
Diagnosis	Chest X-ray, CT scan, Echocardiogram
Differential diagnosis	Partial anomalous pulmonary venous return, Tetralogy of Fallot
Prevention
Treatment	Surgical repair, Medical management
Medication
Prognosis	Varies, can be good with treatment
Frequency	Rare
Deaths

Scimitar syndrome CXR with key

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

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

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

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

• Congenital heart defect
• Pulmonary veins
• Chest radiograph
• Computed tomography (CT) scan
• Surgery

NIH genetic and rare disease info

• NIH info on Scimitar syndrome

Scimitar syndrome is a rare disease.

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