Neonatal pulmonary vascular disease in hypoplastic left heart syndrome

A term neonate with hypoplastic left heart syndrome underwent surgical palliation using the Norwood procedure on day 2 of life. The postoperative course was complicated by recurrent systemic desaturation leading to death. Postmortem histologic examination of lung tissue confirmed the clinical impression of fixed pulmonary vascular disease. Some neonates with a restrictive interatrial communication may have pulmonary vascular disease at birth, which will negatively influence survival irrespective of the type of palliation undertaken.