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Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects

Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associa...

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Autores principales: Ishida, Hidekazu, Maeda, Jun, Uchida, Keiko, Yamagishi, Hiroyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10455332/
https://www.ncbi.nlm.nih.gov/pubmed/37623346
http://dx.doi.org/10.3390/jcdd10080333
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author Ishida, Hidekazu
Maeda, Jun
Uchida, Keiko
Yamagishi, Hiroyuki
author_facet Ishida, Hidekazu
Maeda, Jun
Uchida, Keiko
Yamagishi, Hiroyuki
author_sort Ishida, Hidekazu
collection PubMed
description Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex congenital heart disease (CHD), pulmonary atresia with ventricular septal defect, and aortopulmonary collateral arteries. Some cases of complex CHD, associated with a single ventricle after Fontan operation, show pathological changes in the small peripheral pulmonary arteries and pulmonary vascular resistance similar to those observed in pulmonary arterial hypertension (PAH). This condition is termed as the pediatric pulmonary hypertensive vascular disease (PPHVD). Recent advances in genetics have identified the genes responsible for PAH associated with developmental defects of the heart and lungs, such as TBX4 and SOX17. Targeted therapies for PAH have been developed; however, their effects on PH associated with developmental heart and lung defects remain to be established. Real-world data analyses on the anatomy, pathophysiology, genetics, and molecular biology of unique PPHVD cases associated with developmental defects of the heart and lungs, using nationwide and/or international registries, should be conducted in order to improve the treatments and prognosis of patients with these types of pediatric PH.
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spelling pubmed-104553322023-08-26 Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects Ishida, Hidekazu Maeda, Jun Uchida, Keiko Yamagishi, Hiroyuki J Cardiovasc Dev Dis Review Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex congenital heart disease (CHD), pulmonary atresia with ventricular septal defect, and aortopulmonary collateral arteries. Some cases of complex CHD, associated with a single ventricle after Fontan operation, show pathological changes in the small peripheral pulmonary arteries and pulmonary vascular resistance similar to those observed in pulmonary arterial hypertension (PAH). This condition is termed as the pediatric pulmonary hypertensive vascular disease (PPHVD). Recent advances in genetics have identified the genes responsible for PAH associated with developmental defects of the heart and lungs, such as TBX4 and SOX17. Targeted therapies for PAH have been developed; however, their effects on PH associated with developmental heart and lung defects remain to be established. Real-world data analyses on the anatomy, pathophysiology, genetics, and molecular biology of unique PPHVD cases associated with developmental defects of the heart and lungs, using nationwide and/or international registries, should be conducted in order to improve the treatments and prognosis of patients with these types of pediatric PH. MDPI 2023-08-03 /pmc/articles/PMC10455332/ /pubmed/37623346 http://dx.doi.org/10.3390/jcdd10080333 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Ishida, Hidekazu
Maeda, Jun
Uchida, Keiko
Yamagishi, Hiroyuki
Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
title Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
title_full Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
title_fullStr Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
title_full_unstemmed Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
title_short Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
title_sort unique pulmonary hypertensive vascular diseases associated with heart and lung developmental defects
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10455332/
https://www.ncbi.nlm.nih.gov/pubmed/37623346
http://dx.doi.org/10.3390/jcdd10080333
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