Three-dimensional reconstruction identifies misaligned pulmonary veins as intrapulmonary shunt vessels in alveolar capillary dysplasia

J Pediatr. 2014 Jan;164(1):192-5. doi: 10.1016/j.jpeds.2013.08.035. Epub 2013 Sep 27.


Alveolar capillary dysplasia (ACD) with misalignment of pulmonary veins (MPV) is a lethal neonatal lung disease. Death from ACD/MPV is caused by hypoxia, but the role of the MPV is unknown. Using 3-dimensional reconstruction of ACD/MPV lung tissue, we report that the veins in MPV are intrapulmonary shunt vessels, and speculate that MPV contributes to the poor prognosis.

Keywords: 3-dimensional; 3D; ACD; Alveolar capillary dysplasia; IPSV; Intrapulmonary shunt vessels; MPV; Misalignment of pulmonary veins; PPHN; Persistent pulmonary hypertension of the newborn.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Cadaver
  • Diagnosis, Differential
  • Humans
  • Imaging, Three-Dimensional / methods*
  • Immunohistochemistry / methods*
  • Infant, Newborn
  • Lung / blood supply*
  • Persistent Fetal Circulation Syndrome / diagnosis*
  • Pulmonary Alveoli / abnormalities*
  • Pulmonary Veins / pathology*
  • Reproducibility of Results

Supplementary concepts

  • Alveolar capillary dysplasia