Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins

Thorax. 2015 Jan;70(1):84-5. doi: 10.1136/thoraxjnl-2014-205851. Epub 2014 Jul 21.


Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a lethal neonatal lung disease characterised by severe pulmonary hypertension, abnormal vasculature and intractable hypoxaemia. Mechanisms linking abnormal lung vasculature with severe hypoxaemia in ACD/MPV are unknown. We investigated whether bronchopulmonary anastomoses form right-to-left shunt pathways in ACD/MVP. We studied 2 infants who died of ACD/MPV postmortem with direct injections of coloured ink into the pulmonary artery, bronchial artery and pulmonary veins. Extensive histological evaluations included serial sectioning, immunostaining and 3-dimensional reconstruction demonstrated striking intrapulmonary vascular pathways linking the systemic and pulmonary circulations that bypass the alveolar capillary bed. These data support the role of prominent right-to-left intrapulmonary vascular shunt pathways in the pathophysiology of ACD/MPV.

Keywords: Histology/Cytology; Paediatric interstitial lung disease; Rare lung diseases.

Publication types

  • Letter
  • Research Support, N.I.H., Extramural

MeSH terms

  • Capillaries / pathology*
  • Humans
  • Infant
  • Infant, Newborn
  • Persistent Fetal Circulation Syndrome / pathology*
  • Persistent Fetal Circulation Syndrome / physiopathology
  • Pulmonary Alveoli / abnormalities*
  • Pulmonary Alveoli / pathology
  • Pulmonary Alveoli / physiopathology
  • Pulmonary Artery / pathology*
  • Pulmonary Circulation*
  • Pulmonary Veins / pathology*

Supplementary concepts

  • Alveolar capillary dysplasia