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

19 Citations (Scopus)

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

Fingerprint

Persistent Fetal Circulation Syndrome
Blood Vessels
Infant, Newborn, Diseases
Bronchial Arteries
Ink
Pulmonary Circulation
Pulmonary Veins
Pulmonary Hypertension
Pulmonary Artery
Lung Diseases
Lung
Injections

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins. / Galambos, Csaba; Sims-Lucas, Sunder; Ali, Noorjahan; Gien, Jason; Dishop, Megan K.; Abman, Steven H.

In: Thorax, Vol. 70, No. 1, 17, 01.01.2015, p. 84-85.

Research output: Contribution to journalArticle

Galambos, Csaba ; Sims-Lucas, Sunder ; Ali, Noorjahan ; Gien, Jason ; Dishop, Megan K. ; Abman, Steven H. / Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins. In: Thorax. 2015 ; Vol. 70, No. 1. pp. 84-85.
@article{0cfb3128e9ae42e9bef7ba0185c8c702,
title = "Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins",
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.",
author = "Csaba Galambos and Sunder Sims-Lucas and Noorjahan Ali and Jason Gien and Dishop, {Megan K.} and Abman, {Steven H.}",
year = "2015",
month = "1",
day = "1",
doi = "10.1136/thoraxjnl-2014-205851",
language = "English (US)",
volume = "70",
pages = "84--85",
journal = "Thorax",
issn = "0040-6376",
publisher = "BMJ Publishing Group",
number = "1",

}

TY - JOUR

T1 - Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins

AU - Galambos, Csaba

AU - Sims-Lucas, Sunder

AU - Ali, Noorjahan

AU - Gien, Jason

AU - Dishop, Megan K.

AU - Abman, Steven H.

PY - 2015/1/1

Y1 - 2015/1/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84919767487&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84919767487&partnerID=8YFLogxK

U2 - 10.1136/thoraxjnl-2014-205851

DO - 10.1136/thoraxjnl-2014-205851

M3 - Article

VL - 70

SP - 84

EP - 85

JO - Thorax

JF - Thorax

SN - 0040-6376

IS - 1

M1 - 17

ER -