Level 3 Cardiopulmonary Exercise Testing to Guide Therapeutic Decisions in Non-Severe Pulmonary Hypertension with Lung Disease
Inhaled treprostinil is approved for the treatment of pulmonary hypertension-associated interstitial lung disease (PH-ILD); however, it has not shown significant benefit in patients with a pulmonary vascular resistance (PVR) < 4 WU. As such, treatment for non-severe PH-ILD remains controversial....
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| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2025-07-01
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| Series: | Life |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-1729/15/7/1089 |
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| Summary: | Inhaled treprostinil is approved for the treatment of pulmonary hypertension-associated interstitial lung disease (PH-ILD); however, it has not shown significant benefit in patients with a pulmonary vascular resistance (PVR) < 4 WU. As such, treatment for non-severe PH-ILD remains controversial. A total of 16 patients with non-severe PH-ILD were divided into two groups based on changes in PVR during exercise: a dynamic PVR group (<i>n</i> = 10), characterized by an increase in PVR with exertion, and a static PVR group (<i>n</i> = 6), with no increase in PVR with exercise. The dynamic PVR group received inhaled treprostinil, while the static PVR group was monitored off therapy. Baseline and 16-week follow-up values were compared within each group. At 16 weeks, the dynamic PVR group demonstrated significant improvements in mean 6 min walk distance (6MWD) (+32.5 m, <i>p</i> < 0.05), resting PVR (−1.04 WU, <i>p</i> < 0.05), resting mean pulmonary arterial pressure (mPAP) (−5.8 mmHg, <i>p</i> < 0.05), exercise PVR (−1.7 WU, <i>p</i> < 0.05), exercise mPAP (−13 mmHg, <i>p</i> < 0.05), and estimated right ventricular systolic pressure (−9.2 mmHg, <i>p</i> < 0.05). In contrast, the static PVR group remained clinically stable. These observations suggest that an exercise-induced increase in PVR, identified through Level 3 CPET, may help select patients with non-severe PH-ILD who are more likely to benefit from early initiation of inhaled treprostinil. |
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| ISSN: | 2075-1729 |