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

Csaba Galambos, Sunder Sims-Lucas, Noorjahan Ali, Jason Gien, Megan K. Dishop, Steven H. Abman

Research output: Contribution to journalArticle

22 Scopus citations

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.

Original languageEnglish (US)
Article number17
Pages (from-to)84-85
Number of pages2
JournalThorax
Volume70
Issue number1
DOIs
StatePublished - Jan 1 2015

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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