2020 Volume 36 Issue 2 Pages 152-156
Congenital unilateral pulmonary vein stenosis is an extremely rare condition. Herein, we present a case of congenital unilateral pulmonary stenosis and atresia that we ultimately diagnosed by cardiac catheterization. The patient presented to the hospital with an abnormal shadow on a chest X-ray during a school examination. An aberrantly draining pulmonary vein developed from the atresia, suggesting an anomalous unilateral single pulmonary vein (AUSPV). This is the first report of a patient with congenital pulmonary stenosis with AUSPV. As there was no pulmonary hypertension at diagnosis, the patient was followed up without treatment.