Exercise physiology in pulmonary hypertension patients with and without congenital heart disease

Francesca M. Righini, Anna Apostolo, Pinar B. Heck, Stefania Farina, Alfred Hager, Michele Correale, Roberto Badagliacca, Simone Barbieri, Susanna Sciomer, Piergiuseppe Agostoni

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)86-93
Number of pages8
JournalEuropean Journal of Preventive Cardiology
Volume26
Issue number1
DOIs
Publication statusPublished - Jan 1 2019

Keywords

  • adult congenital heart disease; pulmonary arterial hypertension
  • Cardiopulmonary exercise testing
  • oxygen uptake

ASJC Scopus subject areas

  • Epidemiology
  • Cardiology and Cardiovascular Medicine

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