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Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity

BACKGROUND: Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The pu...

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Autores principales: Alsaied, Tarek, Sleeper, Lynn A., Masci, Marco, Ghelani, Sunil J., Azcue, Nina, Geva, Tal, Powell, Andrew J., Rathod, Rahul H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296022/
https://www.ncbi.nlm.nih.gov/pubmed/30558626
http://dx.doi.org/10.1186/s12968-018-0505-4
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author Alsaied, Tarek
Sleeper, Lynn A.
Masci, Marco
Ghelani, Sunil J.
Azcue, Nina
Geva, Tal
Powell, Andrew J.
Rathod, Rahul H.
author_facet Alsaied, Tarek
Sleeper, Lynn A.
Masci, Marco
Ghelani, Sunil J.
Azcue, Nina
Geva, Tal
Powell, Andrew J.
Rathod, Rahul H.
author_sort Alsaied, Tarek
collection PubMed
description BACKGROUND: Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The purpose of this study is to identify the prevalence and associations of MPBF and to determine the impact of MPBF on exercise capacity after the Fontan operation. METHODS: This retrospective single-center study included all patients after Fontan operation who had maximal cardiopulmonary exercise test (CPET) and CMR with flow measurements of the branch pulmonary arteries. MPBF was defined as > 20% difference in branch pulmonary artery flow. Exercise capacity was measured as percent of predicted oxygen consumption at peak exercise (% predicted VO(2)). Linear and logistic regression models were used to determine univariate and multivariable predictors of exercise capacity and correlates of MPBF, respectively. RESULTS: A total of 147 patients who had CMR between 1999 and 2017 were included (median age at CMR 21.8 years [interquartile range (IQR) 16.5–30.6]) and the median time between CMR and CPET was 2.8 months [IQR 0–13.8]. Fifty-three patients (36%) had MPBF (95% CI 29–45%). The mean % predicted VO(2) was 63 ± 16%. Patients with MPBF had lower mean % predicted VO(2) compared to patients without MPBF (60 ± 14% versus 65 ± 16%, p = 0.04). On multivariable analysis, a lower % predicted VO(2) was independently associated with longer time since Fontan, higher ventricular mass-to-volume ratio, and MPBF. On multivariable analysis, only compression of the branch pulmonary arteries by the ascending aorta or aortic root was associated with MPBF (OR 6.5, 95% CI 5.6–7.4, p < 0.001). CONCLUSION: In patients after the Fontan operation, MPBF is common and is independently associated with lower exercise capacity. MPBF was most likely to be caused by pulmonary artery compression by the aortic root or the ascending aorta. This study identifies MPBF as an important risk factor and as a potential target for therapeutic interventions in this fragile patient population.
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spelling pubmed-62960222018-12-18 Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity Alsaied, Tarek Sleeper, Lynn A. Masci, Marco Ghelani, Sunil J. Azcue, Nina Geva, Tal Powell, Andrew J. Rathod, Rahul H. J Cardiovasc Magn Reson Research BACKGROUND: Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The purpose of this study is to identify the prevalence and associations of MPBF and to determine the impact of MPBF on exercise capacity after the Fontan operation. METHODS: This retrospective single-center study included all patients after Fontan operation who had maximal cardiopulmonary exercise test (CPET) and CMR with flow measurements of the branch pulmonary arteries. MPBF was defined as > 20% difference in branch pulmonary artery flow. Exercise capacity was measured as percent of predicted oxygen consumption at peak exercise (% predicted VO(2)). Linear and logistic regression models were used to determine univariate and multivariable predictors of exercise capacity and correlates of MPBF, respectively. RESULTS: A total of 147 patients who had CMR between 1999 and 2017 were included (median age at CMR 21.8 years [interquartile range (IQR) 16.5–30.6]) and the median time between CMR and CPET was 2.8 months [IQR 0–13.8]. Fifty-three patients (36%) had MPBF (95% CI 29–45%). The mean % predicted VO(2) was 63 ± 16%. Patients with MPBF had lower mean % predicted VO(2) compared to patients without MPBF (60 ± 14% versus 65 ± 16%, p = 0.04). On multivariable analysis, a lower % predicted VO(2) was independently associated with longer time since Fontan, higher ventricular mass-to-volume ratio, and MPBF. On multivariable analysis, only compression of the branch pulmonary arteries by the ascending aorta or aortic root was associated with MPBF (OR 6.5, 95% CI 5.6–7.4, p < 0.001). CONCLUSION: In patients after the Fontan operation, MPBF is common and is independently associated with lower exercise capacity. MPBF was most likely to be caused by pulmonary artery compression by the aortic root or the ascending aorta. This study identifies MPBF as an important risk factor and as a potential target for therapeutic interventions in this fragile patient population. BioMed Central 2018-12-17 /pmc/articles/PMC6296022/ /pubmed/30558626 http://dx.doi.org/10.1186/s12968-018-0505-4 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
Alsaied, Tarek
Sleeper, Lynn A.
Masci, Marco
Ghelani, Sunil J.
Azcue, Nina
Geva, Tal
Powell, Andrew J.
Rathod, Rahul H.
Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity
title Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity
title_full Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity
title_fullStr Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity
title_full_unstemmed Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity
title_short Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity
title_sort maldistribution of pulmonary blood flow in patients after the fontan operation is associated with worse exercise capacity
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296022/
https://www.ncbi.nlm.nih.gov/pubmed/30558626
http://dx.doi.org/10.1186/s12968-018-0505-4
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