Alveolar Capillary Dysplasia as a Cause of Persistent Pulmonary Hypertension

Indian Pediatr. 2015 Nov;52(11):984-6. doi: 10.1007/s13312-015-0759-y.


Background: Persistent pulmonary hypertension (PPHN) in a term or late preterm has varied etiology.

Case characteristics: A late preterm neonate operated for esophageal atresia with tracheo-esophageal fistula was complicated by severe pulmonary hypertension and unable to be weaned off from respiratory support.

Outcome: The neonate expired by 15 weeks of life; diagnosis was made on postmortem lung biopsy.

Message: Alveolarcapillary dysplasia should be considered in a neonate with idiopathic refractory PPHN, if associated with anomalies.

Publication types

  • Case Reports

MeSH terms

  • Esophageal Atresia / etiology*
  • Fatal Outcome
  • Humans
  • Infant, Newborn
  • Male
  • Persistent Fetal Circulation Syndrome* / complications
  • Persistent Fetal Circulation Syndrome* / diagnosis
  • Persistent Fetal Circulation Syndrome* / physiopathology
  • Pulmonary Alveoli / abnormalities*
  • Pulmonary Alveoli / physiopathology

Supplementary concepts

  • Alveolar capillary dysplasia