Persistent pulmonary hypertension of newborn due to congenital capillary alveolar dysplasia

Pediatr Pulmonol. 2005 Oct;40(4):349-53. doi: 10.1002/ppul.20244.


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.

Publication types

  • Case Reports

MeSH terms

  • Capillaries / abnormalities*
  • Female
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Infant, Newborn
  • Lung / ultrastructure
  • Pulmonary Alveoli / blood supply*