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Document type:
Article; Comparative Study; Journal Article; Meta-Analysis
Author(s):
Righini, Francesca M; Apostolo, Anna; Heck, Pinar B; Farina, Stefania; Hager, Alfred; Correale, Michele; Badagliacca, Roberto; Barbieri, Simone; Sciomer, Susanna; Agostoni, Piergiuseppe
Title:
Exercise physiology in pulmonary hypertension patients with and without congenital heart disease.
Abstract:
BACKGROUND: Cardiopulmonary exercise testing allows the assessment of integrative cardiopulmonary response to exercise. AIMS: The aim of the study was to better understand the exercise physiology in pulmonary arterial hypertension related to adult congenital heart disease compared to non-adult congenital heart disease patients by means of cardiopulmonary exercise testing parameters. METHODS: The present is a multicentre retrospective study which includes pulmonary hypertension group 1 and group 4 patients. All subjects underwent full clinical and instrumental evaluation, including cardiopulmonary exercise testing and right heart catheterization. RESULTS: One hundred and sixty-seven pulmonary hypertension patients (93 women and 74 men, 57 adult congenital heart disease and 110 non-adult congenital heart disease) were enrolled. Adult congenital heart disease patients had higher pulmonary pressure (mean pulmonary arterial pressure: 59.8 ± 19.5 mmHg vs 44.6 ± 16.5 mmHg, p < 0.001) and lower pulmonary blood flow (pulmonary blood flow: 3.3 (2.1-4.3) l/min vs 4.5 (3.8-5.4) l/min, p < 0.001). At cardiopulmonary exercise testing they had lower peak oxygen uptake/kg (12.8 ± 3.8 ml/kg/min vs 15.5 ± 4.2 ml/kg/min, p < 0.001) and higher ventilation/carbon dioxide elimination slope (53.2 (43.3-64.8) vs 44.0 (34.6-51.6), p < 0.001). When patients were paired for gender and peak oxygen uptake ( ± 1 ml/kg/min), obtaining 44 pairs, adult congenital heart disease patients had higher pulmonary pressure (mean pulmonary arterial pressure: 58.4 ± 20.2 mmHg vs 42.8 ± 16.8 mmHg, p < 0.001) and ventilation/carbon dioxide elimination slope (51.2 (43.4-63.6) vs 44.9 (35.4-55.1), p = 0.033). CONCLUSIONS: In pulmonary arterial hypertension-adult congenital heart disease patients, pulmonary pressure and ventilation/carbon dioxide elimination slope are higher compared to non-adult congenital heart disease pulmonary hypertension patients, while pulmonary blood flow and peak oxygen uptake are lower. After matching patients for gender and peak oxygen uptake, pulmonary pressure and ventilation/carbon dioxide elimination remain higher in adult congenital heart disease patients suggesting that the long-term adaptation to high pulmonary pressure, hypoxia and low pulmonary blood flow, as well as a persisting shunt has, at least partially, preserved exercise performance of pulmonary arterial hypertension-adult congenital heart disease patients.
Journal title abbreviation:
Eur J Prev Cardiol
Year:
2019
Journal volume:
26
Journal issue:
1
Pages contribution:
86-93
Fulltext / DOI:
doi:10.1177/2047487318809479
Pubmed ID:
http://view.ncbi.nlm.nih.gov/pubmed/30354746
Print-ISSN:
2047-4873
TUM Institution:
Klinik für Kinderkardiologie und angeborene Herzfehler (Prof. Hess)
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