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.
Keywords Sildenafil - Pulmonary hypertension - Pulmonary artery agensis