Alveolar Capillary Dysplasia as a Cause of Persistent Pulmonary Hypertension

Background: Persistent pulmonary hypertension (PPHN) in a term or late preterm has varied etiology. Case characteristics: A late preterm neonate operated for esophageal atresia with tracheo-esophageal fistula was complicated by severe pulmonary hypertension and unable to be weaned off from respiratory support. Outcome: The neonate expired by 15 weeks of life; diagnosis was made on postmortem lung biopsy. Message: Alveolarcapillary dysplasia should be considered in a neonate with idiopathic refractory PPHN, if associated with anomalies.