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.