Scimitar Syndrome with Acquired Cardiac Disease: A Once-in-a-blue-moon Occurrence

Abstract

Abstract Scimitar syndrome (SS) is a rare congenital anomaly characterized by various abnormalities observed in both children and adults, primarily involving partial anomalous pulmonary venous drainage from the right lung to the inferior vena cava and right lung hypoplasia. It is also known by alternative names such as congenital venolobar syndrome, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, and vena cava bronchovascular syndrome. The clinical presentation of this syndrome varies widely, ranging from asymptomatic cases to severe forms. Surgical intervention is typically required for severe cases, and the diagnosis relies predominantly on imaging studies. We present two instances of SS: one involving a patient with underlying rheumatic heart disease scheduled for valve replacement surgery and the other as an incidental finding in an asymptomatic individual, managed through medical means.