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Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension
Despite advances in therapy, outcomes for children with pulmonary hypertension remain poor. We sought to assess the validity of a pediatric-specific functional classification system for pulmonary hypertension (PH) in a heterogeneous population of children with PH diagnosed by echocardiogram or cardi...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4826405/ https://www.ncbi.nlm.nih.gov/pubmed/26843461 http://dx.doi.org/10.1007/s00246-016-1347-1 |
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author | Balkin, Emily Morell Olson, Emma D. Robertson, Laura Adatia, Ian Fineman, Jeffrey R. Keller, Roberta L. |
author_facet | Balkin, Emily Morell Olson, Emma D. Robertson, Laura Adatia, Ian Fineman, Jeffrey R. Keller, Roberta L. |
author_sort | Balkin, Emily Morell |
collection | PubMed |
description | Despite advances in therapy, outcomes for children with pulmonary hypertension remain poor. We sought to assess the validity of a pediatric-specific functional classification system for pulmonary hypertension (PH) in a heterogeneous population of children with PH diagnosed by echocardiogram or cardiac catheterization. A single-center, retrospective study of 65 infants and children with PH was performed. Pediatric Functional Class (FC) at diagnosis, at last visit, and change in FC over time were evaluated for their association with mortality and PH-associated morbidity in univariate, time-to-event, and multivariate regression analyses. Median age at PH diagnosis was 5.3 months (0 days–12.7 years). Twenty-five children (38 %) had idiopathic PH or PH secondary to congenital heart disease, one (2 %) had left heart disease, and 39 (60 %) had PH secondary to respiratory disease. Mortality was 25 % (16/63), primarily in the first year of follow-up. FC at diagnosis was not significantly associated with survival (p = 0.22), but higher FC (more impaired) at last visit (p < 0.001) and change in FC over time (HR 2.3, 95 % confidence interval 1.3–4, p = 0.0003) were associated with mortality. Higher FC at last visit was associated with greater days of hospitalization in the intensive care unit per year (p = 0.006) and history of cardiac arrest (p = 0.012) and syncope (p = 0.02). Although pediatric FC at diagnosis was not predictive of mortality, response to therapy (as assessed by change in FC over time and FC at last visit) was associated with morbidity and mortality in this heterogeneous cohort. Multicenter prospective studies are necessary to further validate these findings. |
format | Online Article Text |
id | pubmed-4826405 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Springer US |
record_format | MEDLINE/PubMed |
spelling | pubmed-48264052016-04-20 Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension Balkin, Emily Morell Olson, Emma D. Robertson, Laura Adatia, Ian Fineman, Jeffrey R. Keller, Roberta L. Pediatr Cardiol Original Article Despite advances in therapy, outcomes for children with pulmonary hypertension remain poor. We sought to assess the validity of a pediatric-specific functional classification system for pulmonary hypertension (PH) in a heterogeneous population of children with PH diagnosed by echocardiogram or cardiac catheterization. A single-center, retrospective study of 65 infants and children with PH was performed. Pediatric Functional Class (FC) at diagnosis, at last visit, and change in FC over time were evaluated for their association with mortality and PH-associated morbidity in univariate, time-to-event, and multivariate regression analyses. Median age at PH diagnosis was 5.3 months (0 days–12.7 years). Twenty-five children (38 %) had idiopathic PH or PH secondary to congenital heart disease, one (2 %) had left heart disease, and 39 (60 %) had PH secondary to respiratory disease. Mortality was 25 % (16/63), primarily in the first year of follow-up. FC at diagnosis was not significantly associated with survival (p = 0.22), but higher FC (more impaired) at last visit (p < 0.001) and change in FC over time (HR 2.3, 95 % confidence interval 1.3–4, p = 0.0003) were associated with mortality. Higher FC at last visit was associated with greater days of hospitalization in the intensive care unit per year (p = 0.006) and history of cardiac arrest (p = 0.012) and syncope (p = 0.02). Although pediatric FC at diagnosis was not predictive of mortality, response to therapy (as assessed by change in FC over time and FC at last visit) was associated with morbidity and mortality in this heterogeneous cohort. Multicenter prospective studies are necessary to further validate these findings. Springer US 2016-02-03 2016 /pmc/articles/PMC4826405/ /pubmed/26843461 http://dx.doi.org/10.1007/s00246-016-1347-1 Text en © The Author(s) 2016 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. |
spellingShingle | Original Article Balkin, Emily Morell Olson, Emma D. Robertson, Laura Adatia, Ian Fineman, Jeffrey R. Keller, Roberta L. Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension |
title | Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension |
title_full | Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension |
title_fullStr | Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension |
title_full_unstemmed | Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension |
title_short | Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension |
title_sort | change in pediatric functional classification during treatment and morbidity and mortality in children with pulmonary hypertension |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4826405/ https://www.ncbi.nlm.nih.gov/pubmed/26843461 http://dx.doi.org/10.1007/s00246-016-1347-1 |
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