Breathless at the Point of a Sword

Abstract

Context: Scimitar syndrome is a congenital anomaly of pulmonary venous return where right pulmonary artery drains into right side other heart, instead of the left side, causing pulmonary hypertension resulting in shortness of breath, recurrent lower respiratory tract infections, chest pain, and fatigue. Early diagnosis and surgical intervention would correct this congenital anomaly reducing morbidity and complications in otherwise healthy young patients. Case Report: We present a case of an 18-year-old female who presented with exertional shortness of breath, fatigue, and recurrent lower respiratory tract infections. She had unremarkable physical examination but chest x-ray showed an abnormal opacity next to right heart border. Computed tomography (CT) chest was performed that showed possible scimitar syndrome. Transesophageal echocardiogram (TEE) and right heart catheterization (RHC) confirmed the diagnosis. Conclusion: Scimitar syndrome is a very rare congenital anomaly of pulmonary venous return. It is usually diagnosed in early childhood but the diagnosis may be delayed until later in adulthood. The consequences are pulmonary hypertension, right-sided heart failure, and frequent pulmonary infections resulting in increased morbidity, mortality, and frequent doctor visits for otherwise healthy young patients.