Abstract

Scimitar Syndrome: A Rare Case Report

Scimitar syndrome is a rare congenital anomaly. It is also known as hypogenetic lung syndrome. It is essentially a combination of pulmonary hypoplasia and Partial Anomalous Pulmonary Venous Return (PAPVR). There are manifestations of heart failure due to associated congenital heart disease in infants. Most of the adults are asymptomatic but may have exertional dyspnoea or recurrent respiratory infection. It almost exclusively occurs on the right side. It is diagnosed by transthoracic/transesophageal echocardiography or angiography. CT or MRI angiography also helps in the diagnosis. Surgical correction is indicated in the presence of significant left to right shunting and pulmonary hypertension. Creation of an intra-atrial baffle to redirect the pulmonary venous return into the left atrium. Otherwise, the anomalous vein can be re-implanted directly into the left atrium. Thrombosis, stenosis, and occlusion of the scimitar vein and its deviation are the complications of the surgery. This case report emphasizes the importance of a thorough clinical and echocardiographic examination to avoid the diagnosis of this rare congenital anomaly.

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