Congenital pulmonary venous stenosis is an uncommon congenital heart defect and is a consequence of failed incorporation of the common right or left pulmonary vein into the left atrium during embryologic development. Clinical manifestations include progressive exertional dyspnea, cough, chest pain, and hemoptysis. In this report, we describe a 37-year-old woman with known congenital unilateral pulmonary venous stenosis and a unilateral hypoplastic right pulmonary artery who presented with recurrent episodes of hemoptysis, requiring multiple catheter-based interventions, and symptomatic pleural effusions, requiring multiple large-volume thoracenteses. The patient underwent successful video-assisted thoracoscopic pneumonectomy with resolution of symptoms.
