Abstract

Scimitar syndrome is a rare birth defect characterized by abnormal right pulmonary venous return to the inferior vena cava. It can be partial (PAPVR) or total (TAPVR). Anomalous right-sided pulmonary veins might drain into the superior vena cava, azygos vein, inferior vena cava, or less commonly into the right atrium. Two clinical forms can be distinguished: an infantile form with severe pulmonary arterial hypertension (PAH) and heart failure, with a poor prognosis; and a pediatric or adult form without PAH, well tolerated and with a good prognosis. We report a case of accidentally discovered scimitar syndrome in a 66-year-old patient with dyspnea on exertion as a symptom. The diagnosis was suspected on the chest x-ray and confirmed on a CT scan which showed a single large right pulmonary vein emptying into the right atrium associated with dextro cardia and pulmonary sequestration. Our patient was asymptomatic, apart from stage 1 dyspnea, that's why therapeutic abstention and monitoring were indicated for her.