Scimitar Syndrome: Late Presentation and Conservative Management

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital malformation. The infracardiac variant with the right lobe of the lung draining to the inferior vena cava (IVC) is called Scimitar syndrome. The infantile subtype presents before one year of age and the adult variant is also usually diagnosed in childhood. A 70-year-old woman presented with worsening shortness of breath. An echocardiogram suggested severe pulmonary hypertension that was confirmed by right heart catheterization. A computed tomography (CT) without contrast revealed an anomalous vein from the right upper lobe suggestive of Scimitar syndrome. The patient did not have any other associated congenital heart defects (CHD) (incomplete Scimitar syndrome). A surgical treatment approach was avoided due to the incomplete nature of the Scimitar syndrome. Incomplete Scimitar syndrome may present later and with less severity than the typical Scimitar syndrome with left to right shunting occurring only in the lung and may be managed nonsurgically.