A 19 year-old woman who underwent corrective surgery for an atrial septal defect (ASD) and ventricular septal defect (VSD) 12 years previously presented with pulmonary hypertension. Cardiac catheterization showed a fistula between the right subclavian artery and vena jugularis interna. In this case, a rare example of secondary pulmonary hypertension due to acquired arteriovenous fistula after cardiac surgery is presented.