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Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins
  1. Csaba Galambos1,2,
  2. Sunder Sims-Lucas3,
  3. Noorjahan Ali2,4,
  4. Jason Gien2,4,
  5. Megan K Dishop1,
  6. Steven H Abman2,4
  1. 1Department of Pathology, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado, USA
  2. 2Pediatric Heart Lung Center, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado, USA
  3. 3Division of Nephrology, Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA
  4. 4Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado, USA
  1. Correspondence to Dr Csaba Galambos, Department of Pathology and Laboratory Medicine, Children's Hospital Colorado, University School of Medicine of Colorado, 13132 East 16th Avenue, Aurora, CO 80045, USA; csaba.galambos{at}childrenscolorado.org

Abstract

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.

  • Paediatric interstitial lung disease
  • Rare lung diseases
  • Histology/Cytology

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