Introduction: In children, pulmonary hypertension (PH) is usually associated with underlying cardiac or lung disease. Bronchopulmonary dysplasia (BPD) is a chronic lung disease that develops in a preterm infants. Methods: We report the case of a preterm infant with gestational age of 24 weeks and extremely low birth weight of 580g, diagnosed with BPD. At 6-months of age the infant was admitted in out center for respira-tory distress. Echocardiography showed severe PH, a small atrial septal defect, bidirectional shunting, and a small patent ductus arteriosus, bidirectional shunting. Chest CT scan showed ground glass opacities. Right heart catheterization revealed a mean pulmonary arterial pressure of 52mmHg, and pulmonary vascular resistance (PVR) of 19.39WU/m2, no reactivity to NO. Specific therapy with Sildenafil was started.
Results: Echocardiographic exam 1 year after initiation of Sildenafil showed PH progression, with right-to-left shunt both at atrial and ductal level. Bosentan therapy was associated.
Conclusions: This case confirms that preterm infants with BPD are at risk for PH. Persistent echocardiogra-phic evidence of PH beyond the first few months of life has been associated with poor prognosis. The severity of pulmonary vascular disease requires the escalation of specific therapy.