Ventricular septal defect with right pulmonary agenesis and left bronchial stenosis

We report a case of ventricular septal defect (VSD) with right pulmonary agenesis and left bronchial stenosis. Delivery of a male infant was uneventful. Birth weight was 3,050g. At 12 days of age, he presented himself with tachypnea and wheezing. Dextrocardia was noted on a chest X-ray. Computed tomography (CT) of the chest showed right pulmonary agenesis and severe narrowing of the left main bronchus. An echocardiogram showed VSD, patent ductus arteriosus (PDA) and pulmonary hypertension (PH). At 22 days of age, he was put on ventilator. At 1 month of age, pulmonary artery banding and division of PDA were performed through median sternotomy. At 5 months of age, weighing 5.0 kg, the VSD was closed with a Dacron patch through median sternotomy. At 6 months of age, tracheostomy was necessitated. At 1-year-old, he became free from ventilator.