A lobe lost and a vein misplaced: an adult’s tale of scimitar syndrome

Abstract Background Scimitar syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return (PAPVR), most often involving drainage of the right pulmonary veins into the inferior vena cava (IVC) instead of the left atrium. It is commonly associated with right lung hypoplasia, dextroposition of the heart, and other cardiovascular or bronchopulmonary abnormalities. While usually diagnosed in infancy or childhood, adult presentations are uncommon and often manifest with vague or non-specific respiratory symptoms. Case presentation We report the case of a 60-year-old female who presented with intermittent dyspnea and was found to have right upper lobe agenesis, middle lobe aplasia, and anomalous right pulmonary venous drainage to the IVC. The diagnosis of Scimitar syndrome was confirmed based on imaging studies. This case is unique in highlighting the diagnostic complexity and subtlety of adult presentations, in which anatomical abnormalities may be overlooked or misattributed to other causes of dyspnea. Conclusion Scimitar syndrome, though typically diagnosed early in life, should be considered in adults presenting with unexplained respiratory symptoms and radiological anomalies. Awareness of this rare condition and its variable presentations is crucial for timely diagnosis and management. We also present a concise review of the available literature outlining diagnostic approaches and therapeutic considerations in adult cases of Scimitar syndrome.