Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension
Introduction Despite shared features with pulmonary arterial hypertension, acute vasoreactivity in pulmonary hypertension with interstitial lung disease (PH-ILD) is not well characterised, including its potential ability to predict therapeutic outcomes. We sought to determine whether acute vasoreact...
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| Format: | Article |
| Language: | English |
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European Respiratory Society
2024-12-01
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| Series: | ERJ Open Research |
| Online Access: | http://openres.ersjournals.com/content/10/6/00201-2024.full |
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| author | Eileen M. Harder Farbod N. Rahaghi Jane A. Leopold David M. Systrom George R. Washko Aaron B. Waxman |
| author_facet | Eileen M. Harder Farbod N. Rahaghi Jane A. Leopold David M. Systrom George R. Washko Aaron B. Waxman |
| author_sort | Eileen M. Harder |
| collection | DOAJ |
| description | Introduction
Despite shared features with pulmonary arterial hypertension, acute vasoreactivity in pulmonary hypertension with interstitial lung disease (PH-ILD) is not well characterised, including its potential ability to predict therapeutic outcomes. We sought to determine whether acute vasoreactivity in PH-ILD to oxygen (O2) and inhaled nitric oxide (iNO) predicts inhaled treprostinil (iTre) outcomes.
Materials and methods
In this retrospective cohort analysis, we identified treatment-naive PH-ILD patients with vasoreactivity testing using O2 and O2+iNO. 6-month iTre outcome was assessed. “iTre improvement” required fulfilment of criteria on objective assessment without clinical worsening. “iTre failure” was defined by lack of objective improvement or a clinical worsening event.
Results
Among 75 PH-ILD patients, mean pulmonary arterial pressure (mPAP) decreased by −3 mmHg (−12.6%) and pulmonary vascular resistance (PVR) by −1.3 WU (−23.7%) with O2+iNO. With O2+iNO, mPAP decreased ≥10 mmHg to <40 mmHg in four patients (5.3%) and 23 (30.7%) had ≥20% reduction in mPAP and PVR. Among 33 iTre-treated patients, there were 13 improvements and 20 failures. The microvascular response, measured by distensibility, to O2 alone versus O2+iNO correlated with 6-month iTre outcome. Patients with 6-month iTre improvement had large relative distensibility increases with O2+iNO (versus failure, 76.0% versus 15.3%, p=0.004). Conversely, iTre failure was associated with increased distensibility with O2 alone (versus improvement, 26.8% versus −3.9%, p=0.045).
Conclusions
In PH-ILD, the microvascular response to O2 versus O2+iNO testing was associated with 6-month iTre outcome, likely reflecting the differential contributions of hypoxic vasoconstriction and remodelling. Acute vasoreactivity may inform therapeutic decision-making in PH-ILD. |
| format | Article |
| id | doaj-art-552df4ac647443488820f997365df9e9 |
| institution | Kabale University |
| issn | 2312-0541 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | ERJ Open Research |
| spelling | doaj-art-552df4ac647443488820f997365df9e92025-01-14T09:50:21ZengEuropean Respiratory SocietyERJ Open Research2312-05412024-12-0110610.1183/23120541.00201-202400201-2024Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertensionEileen M. Harder0Farbod N. Rahaghi1Jane A. Leopold2David M. Systrom3George R. Washko4Aaron B. Waxman5 Brigham and Women's Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, USA Brigham and Women's Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, USA Brigham and Women's Hospital, Division of Cardiovascular Medicine, Boston, MA, USA Brigham and Women's Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, USA Brigham and Women's Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, USA Brigham and Women's Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, USA Introduction Despite shared features with pulmonary arterial hypertension, acute vasoreactivity in pulmonary hypertension with interstitial lung disease (PH-ILD) is not well characterised, including its potential ability to predict therapeutic outcomes. We sought to determine whether acute vasoreactivity in PH-ILD to oxygen (O2) and inhaled nitric oxide (iNO) predicts inhaled treprostinil (iTre) outcomes. Materials and methods In this retrospective cohort analysis, we identified treatment-naive PH-ILD patients with vasoreactivity testing using O2 and O2+iNO. 6-month iTre outcome was assessed. “iTre improvement” required fulfilment of criteria on objective assessment without clinical worsening. “iTre failure” was defined by lack of objective improvement or a clinical worsening event. Results Among 75 PH-ILD patients, mean pulmonary arterial pressure (mPAP) decreased by −3 mmHg (−12.6%) and pulmonary vascular resistance (PVR) by −1.3 WU (−23.7%) with O2+iNO. With O2+iNO, mPAP decreased ≥10 mmHg to <40 mmHg in four patients (5.3%) and 23 (30.7%) had ≥20% reduction in mPAP and PVR. Among 33 iTre-treated patients, there were 13 improvements and 20 failures. The microvascular response, measured by distensibility, to O2 alone versus O2+iNO correlated with 6-month iTre outcome. Patients with 6-month iTre improvement had large relative distensibility increases with O2+iNO (versus failure, 76.0% versus 15.3%, p=0.004). Conversely, iTre failure was associated with increased distensibility with O2 alone (versus improvement, 26.8% versus −3.9%, p=0.045). Conclusions In PH-ILD, the microvascular response to O2 versus O2+iNO testing was associated with 6-month iTre outcome, likely reflecting the differential contributions of hypoxic vasoconstriction and remodelling. Acute vasoreactivity may inform therapeutic decision-making in PH-ILD.http://openres.ersjournals.com/content/10/6/00201-2024.full |
| spellingShingle | Eileen M. Harder Farbod N. Rahaghi Jane A. Leopold David M. Systrom George R. Washko Aaron B. Waxman Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension ERJ Open Research |
| title | Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension |
| title_full | Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension |
| title_fullStr | Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension |
| title_full_unstemmed | Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension |
| title_short | Vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension |
| title_sort | vasoreactivity and inhaled treprostinil response in interstitial lung disease pulmonary hypertension |
| url | http://openres.ersjournals.com/content/10/6/00201-2024.full |
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