Scimitar Syndrome: Unveiling the Complexities of a rare Congenital Cardiopulmonary Anomaly.

Abstract

Scimitar syndrome is a rare congenital anomaly characterised by anomalous pulmonary venous drainage of the right lung into the inferior vena cava, often accompanied by right lung hypoplasia and dextroposition of the heart. Here, we present the case of a 24-year-old female with scimitar syndrome and a history of asthma who presented with acute exacerbation of asthma. A chest X-ray revealed opacification of the right hemithorax, and a CT angiography revealed congenital hypoplasia of the right lung and dextrocardia. This report provides a detailed description of scimitar syndrome to spread awareness of this rare diagnosis and highlights the unique and various constellations of anatomical abnormalities seen in scimitar syndrome that make diagnosis difficult.

Learning points: Scimitar syndrome is an extremely rare congenital cardiopulmonary anomaly and diagnostic challenge, with key features of partial or complete anomalous venous drainage of the right lung into the inferior vena cava. It exhibits varying degrees of right lung and pulmonary artery hypoplasia, dextrocardia and abnormal arterial supply to the ipsilateral lung.The adult form of this rare syndrome may remain underdiagnosed for years due to its less severe clinical presentation or incidental findings, such as recurrent pulmonary infections or unexplained dyspnoea.