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Congenital Pulmonary Vein Stenosis is a rare condition that presents in infancy with refractory wheezing from pulmonary venous obstruction. It should be considered when more common causes of wheezing have been excluded. Our case, a 6-month-old boy presented with a progressive course. His echocardiogram demonstrated stenosis of the right pulmonary veins which was defined and followed on cardiac computed tomography.
LEARNING OBJECTIVES: Refractory wheezing is recurrent or prolonged wheezing that is difficult to treat and unresponsive to therapy. In infancy, this type of wheezing should elicit investigation for less common causes. Due to the close relationship between the circulatory and respiratory systems, cardiac anomalies should be considered. Our case of congenital pulmonary vein stenosis presented with refractory wheezing from pulmonary venous obstruction. Unfortunately, this disorder has an unrelenting course. Even with early diagnosis and current surgical management, the outcome is poor.