Alveolar Capillary Dysplasia: A Lethal Developmental Lung Malformation

Alveolar capillary dysplasia (ACD) is a recently recognized lethal lung malformation. ACD is a rare congenital disorder of pulmonary vascular development associated with unremitting hypoxemia and respiratory failure. This paper reviews the incidence, pathophysiology and histopathology, clinical manifestations, diagnosis, and treatment of affected newborns and proposes an algorithmic approach to management of infants who present with unremitting persistent pulmonary hypertension of the newborn.