Scimitar Syndrome is a rare congenital disorder and a variant of partial anomalous pulmonary venous connection (PAPVC) in which part or even the entire right lung is drained by right pulmonary veins that connect anomalously to the Inferior Vena Cava (IVC). It has various presentations including exertional dyspnea, recurrent chest infection, pulmonary artery hypertension, and hemoptysis. The initial diagnosis of PAPVC may be made by echocardiography and is typically confirmed by magnetic resonance imaging, computed tomography, or cardiac catheterization.We report a 69-year-old man with progressive dyspnea on exertion associated with palpitations of five years of evolution. The patient was diagnosed with cardiomyopathy, pulmonary hypertension and was started on treatment with anticoagulation, digoxin, and metoprolol for his atrial fibrillation. Despite the treatment, the dyspnea did not improve. The patient underwent cardiac catheterization, where the anomalous venous drainage was confirmed. Scimitar syndrome was corrected by surgical intervention with complete resolution of symptoms.
Keywords: Dyspnea; Hemoptysis; Pulmonary Arterial Hypertension; Scimitar Syndrome.
© 2020 The Authors.