Sildenafil in pulmonary hypertension secondary to unilateral agenesis of pulmonary artery.

Unilateral agenesis of the pulmonary artery (PA) is a rare anomaly that can lead to pulmonary hypertension (PHTN). We report the case of a 10-month-old female with agenesis of the left PA, ventricular septal defect, and patent ductus arteriosus who developed PHTN precluding surgical repair. The use of sildenafil decreased PA pressure and normalized pulmonary vascular resistance, after which surgery was successfully performed.