UNILATERAL PULMONARY-HYPERTENSION AS A RESULT OF CHRONIC HIGH FLOW TO ONE LUNG

The patient presented is a 27-month-old male with complex congenital heart disease consisting of severe left ventricular outflow tract obstruction and ventricular septal defect who had undergone a pulmonary trunk-to-aorta graft and a pulmonary artery banding procedure as a neonate. Sometime after this repair, but at least 15 months prior to presentation to this institution for placement of an oarta homograft, the pulmonary trunk band apparently slipped and migrated over the right pulmonary artery, severely limiting blood flow to the right lung and increasing flow to the left. Severe pulmonary hypertension developed, with a main pulmonary artery pressure of 94/53 mm Hg. We present clinical and radiographic evidence that the resulting chronic high blood flow and pressure in the left lung ultimately resulted in hypoperfusion of that lung, presumably secondary to chronic vascular changes with greatly increased vascular resistance. Upon surgical repair and removal of the constrictive band from the previously banded right PA, blood flow was increased to the low resistance right lung causing right-side unilateral pulmonary edema, ventilation/perfusion mismatching, and severe hypoxemia. Perfusion studies documented that less than 10% of blood was directed to the left lung, with greater than 90% to the right. Perfusion studies 9 months postoperatively continued to demonstrate minimal blood flow to the left lung. Discussion focuses on the effects of mechanical forces and the interaction with hypoxia in causing pulmonary vascular remodeling.