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Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease
Pulmonary hypertension associated with congenital heart disease (CHD-PH) encompasses different conditions confounded by the left-to-right shunt, left heart obstruction, ventricular dysfunction, hypoxia due to airway obstruction, dysplasia/hypoplasia of the pulmonary vasculature, pulmonary vascular o...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9614099/ https://www.ncbi.nlm.nih.gov/pubmed/36313863 http://dx.doi.org/10.3389/fped.2022.1011631 |
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author | Muneuchi, Jun Ezaki, Hiroki Sugitani, Yuichiro Watanabe, Mamie |
author_facet | Muneuchi, Jun Ezaki, Hiroki Sugitani, Yuichiro Watanabe, Mamie |
author_sort | Muneuchi, Jun |
collection | PubMed |
description | Pulmonary hypertension associated with congenital heart disease (CHD-PH) encompasses different conditions confounded by the left-to-right shunt, left heart obstruction, ventricular dysfunction, hypoxia due to airway obstruction, dysplasia/hypoplasia of the pulmonary vasculature, pulmonary vascular obstructive disease, and genetic variations of vasoactive mediators. Pulmonary input impedance consists of the pulmonary vascular resistance (Rp) and capacitance (Cp). Rp is calculated as the transpulmonary pressure divided by the pulmonary cardiac output, whereas Cp is calculated as the pulmonary stroke volume divided by the pulmonary arterial pulse pressure. The plots of Rp and Cp demonstrate a unique hyperbolic relationship, namely, the resistor–capacitor coupling curve, which represents the pulmonary vascular condition. The product of Rp and Cp is the exponential pressure decay, which refers to the time constant. Alterations in Cp are more considerable in CHD patients at an early stage of developing pulmonary hypertension or with excessive pulmonary blood flow due to a left-to-right shunt. The importance of Cp has gained attention because recent reports have shown that low Cp potentially reflects poor prognosis in patients with CHD-PH and idiopathic pulmonary hypertension. It is also known that Cp levels decrease in specific populations, such as preterm infants and trisomy 21. Therefore, both Rp and Cp should be individually evaluated in the management of children with CHD-PH who have different disease conditions. |
format | Online Article Text |
id | pubmed-9614099 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-96140992022-10-29 Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease Muneuchi, Jun Ezaki, Hiroki Sugitani, Yuichiro Watanabe, Mamie Front Pediatr Pediatrics Pulmonary hypertension associated with congenital heart disease (CHD-PH) encompasses different conditions confounded by the left-to-right shunt, left heart obstruction, ventricular dysfunction, hypoxia due to airway obstruction, dysplasia/hypoplasia of the pulmonary vasculature, pulmonary vascular obstructive disease, and genetic variations of vasoactive mediators. Pulmonary input impedance consists of the pulmonary vascular resistance (Rp) and capacitance (Cp). Rp is calculated as the transpulmonary pressure divided by the pulmonary cardiac output, whereas Cp is calculated as the pulmonary stroke volume divided by the pulmonary arterial pulse pressure. The plots of Rp and Cp demonstrate a unique hyperbolic relationship, namely, the resistor–capacitor coupling curve, which represents the pulmonary vascular condition. The product of Rp and Cp is the exponential pressure decay, which refers to the time constant. Alterations in Cp are more considerable in CHD patients at an early stage of developing pulmonary hypertension or with excessive pulmonary blood flow due to a left-to-right shunt. The importance of Cp has gained attention because recent reports have shown that low Cp potentially reflects poor prognosis in patients with CHD-PH and idiopathic pulmonary hypertension. It is also known that Cp levels decrease in specific populations, such as preterm infants and trisomy 21. Therefore, both Rp and Cp should be individually evaluated in the management of children with CHD-PH who have different disease conditions. Frontiers Media S.A. 2022-10-14 /pmc/articles/PMC9614099/ /pubmed/36313863 http://dx.doi.org/10.3389/fped.2022.1011631 Text en © 2022 Muneuchi, Ezaki, Sugitani and Watanabe. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Muneuchi, Jun Ezaki, Hiroki Sugitani, Yuichiro Watanabe, Mamie Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
title | Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
title_full | Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
title_fullStr | Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
title_full_unstemmed | Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
title_short | Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
title_sort | comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9614099/ https://www.ncbi.nlm.nih.gov/pubmed/36313863 http://dx.doi.org/10.3389/fped.2022.1011631 |
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