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Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development
OBJECTIVES: In congenital heart malformations with pulmonary stenosis to atresia an abnormal lateral ductus arteriosus to left pulmonary artery connection can lead to a localised narrowing (pulmonary ductal coarctation) or even interruption We investigated embryonic remodelling and pathogenesis of t...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7228067/ https://www.ncbi.nlm.nih.gov/pubmed/32413023 http://dx.doi.org/10.1371/journal.pone.0228478 |
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author | Gittenberger-de Groot, Adriana C. Peterson, Joshua C. Wisse, Lambertus J. Roest, Arno A. W. Poelmann, Robert E. Bökenkamp, Regina Elzenga, Nynke J. Hazekamp, Mark Bartelings, Margot M. Jongbloed, Monique R. M. DeRuiter, Marco C. |
author_facet | Gittenberger-de Groot, Adriana C. Peterson, Joshua C. Wisse, Lambertus J. Roest, Arno A. W. Poelmann, Robert E. Bökenkamp, Regina Elzenga, Nynke J. Hazekamp, Mark Bartelings, Margot M. Jongbloed, Monique R. M. DeRuiter, Marco C. |
author_sort | Gittenberger-de Groot, Adriana C. |
collection | PubMed |
description | OBJECTIVES: In congenital heart malformations with pulmonary stenosis to atresia an abnormal lateral ductus arteriosus to left pulmonary artery connection can lead to a localised narrowing (pulmonary ductal coarctation) or even interruption We investigated embryonic remodelling and pathogenesis of this area. MATERIAL AND METHODS: Normal development was studied in WntCre reporter mice (E10.0–12.5) for neural crest cells and Nkx2.5 immunostaining for second heart field cells. Data were compared to stage matched human embryos and a VEGF120/120 mutant mouse strain developing pulmonary atresia. RESULTS: Normal mouse and human embryos showed that the mid-pharyngeal endothelial plexus, connected side-ways to the 6(th) pharyngeal arch artery. The ventral segment formed the proximal pulmonary artery. The dorsal segment (future DA) was solely surrounded by neural crest cells. The ventral segment had a dual outer lining with neural crest and second heart field cells, while the distal pulmonary artery was covered by none of these cells. The asymmetric contribution of second heart field to the future pulmonary trunk on the left side of the aortic sac (so-called pulmonary push) was evident. The ventral segment became incorporated into the pulmonary trunk leading to a separate connection of the left and right pulmonary arteries. The VEGF120/120 embryos showed a stunted pulmonary push and a variety of vascular anomalies. SUMMARY: Side-way connection of the DA to the left pulmonary artery is a congenital anomaly. The primary problem is a stunted development of the pulmonary push leading to pulmonary stenosis/atresia and a subsequent lack of proper incorporation of the ventral segment into the aortic sac. Clinically, the aberrant smooth muscle tissue of the ductus arteriosus should be addressed to prohibit development of severe pulmonary ductal coarctation or even interruption of the left pulmonary artery. |
format | Online Article Text |
id | pubmed-7228067 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Public Library of Science |
record_format | MEDLINE/PubMed |
spelling | pubmed-72280672020-06-01 Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development Gittenberger-de Groot, Adriana C. Peterson, Joshua C. Wisse, Lambertus J. Roest, Arno A. W. Poelmann, Robert E. Bökenkamp, Regina Elzenga, Nynke J. Hazekamp, Mark Bartelings, Margot M. Jongbloed, Monique R. M. DeRuiter, Marco C. PLoS One Research Article OBJECTIVES: In congenital heart malformations with pulmonary stenosis to atresia an abnormal lateral ductus arteriosus to left pulmonary artery connection can lead to a localised narrowing (pulmonary ductal coarctation) or even interruption We investigated embryonic remodelling and pathogenesis of this area. MATERIAL AND METHODS: Normal development was studied in WntCre reporter mice (E10.0–12.5) for neural crest cells and Nkx2.5 immunostaining for second heart field cells. Data were compared to stage matched human embryos and a VEGF120/120 mutant mouse strain developing pulmonary atresia. RESULTS: Normal mouse and human embryos showed that the mid-pharyngeal endothelial plexus, connected side-ways to the 6(th) pharyngeal arch artery. The ventral segment formed the proximal pulmonary artery. The dorsal segment (future DA) was solely surrounded by neural crest cells. The ventral segment had a dual outer lining with neural crest and second heart field cells, while the distal pulmonary artery was covered by none of these cells. The asymmetric contribution of second heart field to the future pulmonary trunk on the left side of the aortic sac (so-called pulmonary push) was evident. The ventral segment became incorporated into the pulmonary trunk leading to a separate connection of the left and right pulmonary arteries. The VEGF120/120 embryos showed a stunted pulmonary push and a variety of vascular anomalies. SUMMARY: Side-way connection of the DA to the left pulmonary artery is a congenital anomaly. The primary problem is a stunted development of the pulmonary push leading to pulmonary stenosis/atresia and a subsequent lack of proper incorporation of the ventral segment into the aortic sac. Clinically, the aberrant smooth muscle tissue of the ductus arteriosus should be addressed to prohibit development of severe pulmonary ductal coarctation or even interruption of the left pulmonary artery. Public Library of Science 2020-05-15 /pmc/articles/PMC7228067/ /pubmed/32413023 http://dx.doi.org/10.1371/journal.pone.0228478 Text en © 2020 Gittenberger-de Groot et al http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Research Article Gittenberger-de Groot, Adriana C. Peterson, Joshua C. Wisse, Lambertus J. Roest, Arno A. W. Poelmann, Robert E. Bökenkamp, Regina Elzenga, Nynke J. Hazekamp, Mark Bartelings, Margot M. Jongbloed, Monique R. M. DeRuiter, Marco C. Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
title | Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
title_full | Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
title_fullStr | Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
title_full_unstemmed | Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
title_short | Pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
title_sort | pulmonary ductal coarctation and left pulmonary artery interruption; pathology and role of neural crest and second heart field during development |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7228067/ https://www.ncbi.nlm.nih.gov/pubmed/32413023 http://dx.doi.org/10.1371/journal.pone.0228478 |
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