Persistent pulmonary hypertension of newborn due to congenital capillary alveolar dysplasia

Congenital alveolar capillary dysplasia is a rare and fatal cause of pulmonary hypertension in neonates. We report on a term baby with severe pulmonary hypertension unresponsive to high-frequency ventilation and nitric oxide. A diagnosis of alveolar capillary dysplasia was established on autopsy. We review the literature regarding pathophysiology, clinical presentations, associated malformations, and treatment trials.