Persistent pulmonary hypertension of the newborn associated with pulmonaryatresia and intact interventricular septum

Neonates with pulmonary atresia and intact interventricular septum (PAIVS) do not have pulmonary vascular disease secondary to their heart abnormality . Persistent pulmonary hypertension of the newborn has not been described i n association with this condition. The ease is reported of a female neonate born with PAIVS, who preoperatively had no clinical evidence or any risk f actors for persistent pulmonary hypertension of the newborn, but whose post operative course was highly suggestive of persistent pulmonary hypertension ; necropsy confirmed the features of pulmonary vascular disease.