Obstructive Ventilatory Defects in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
- 1Department of Medicine V, LMU University Hospital, LMU Munich, Comprehensive Pneumology Center (CPC-M), Member of the German Center for Lung Research (DZL), 81377 Munich, Germany
- 2Division of Rheumatology and Clinical Immunology, Department of Medicine IV, LMU Medizin, LMU Munich, 80336 Munich, Germany
Abstract
Background/Objectives: Patients with pulmonary arterial hypertension (PAH) may display obstructive ventilatory defects outside of defined obstructive lung disease. However, its pathogenesis and prognostic relevance remain unknown. Similar data have emerged for patients with chronic thromboembolic pulmonary hypertension (CTEPH), but there is no description in a real-world European cohort. Methods: We performed a single-center, retrospective, cross-sectional study of patients with PAH (n = 46) and CTEPH (n = 45) at an academic PH center. Data were collected on pulmonary function tests, DLCO, right heart catheterization results, pre-existing diagnoses of asthma and chronic obstructive pulmonary disease (COPD), current medications, and adverse outcomes. Results: Patients with PAH and CTEPH showed a reduced FVC (81% for both groups), FEV1 (74 and 75%) and DLCO (54 and 53%). A previous diagnosis of COPD was documented in 11% of patients, and asthma in 11% of patients with PAH and 4.5% of patients with CTEPH respectively. Even after excluding patients with asthma or COPD, ventilatory obstruction was still present in at least 22% of patients and 16% were being treated with long-acting bronchodilators or inhaled corticosteroids. Of these patients, those who had adverse outcomes (active listing for lung transplantation, lung transplantation or death) had a significantly lower FVC (68 vs. 83%, p = 0.026), FEV1 (61 vs. 79%, p = 0.01) and MEF50 (38 vs. 59%, p = 0.021). All patients with adverse outcomes had a mean expiratory flow at 50% vital capacity (MEF50) < 60%, as opposed to 57% of those without an adverse event, however MEF50 did not predict adverse outcomes after adjusting for FVC. Conclusions: Ventilatory obstruction is common in patients with PAH and CTEPH even when excluding patients with asthma/COPD or a history of smoking. Symptomatic treatment with bronchodilators is common even without these comorbidities. Patients with adverse outcomes had lower FEV1, FVC and MEF50, but the functional relevance of the latter remains to be explored.
Related
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BibTeX
@article{sonneck2026obstructive,
title = {{Obstructive Ventilatory Defects in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension}},
author = {Torben Sonneck and Michaela Barnikel and Paola Arnold and Gabriela Leuschner and Amanda Tufman and Jürgen Behr},
journal = {Journal of Clinical Medicine},
year = {2026},
month = nov,
doi = {10.3390/jcm15218574},
url = {https://www.mdpi.com/2077-0383/15/21/8574},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Obstructive Ventilatory Defects in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
AU - Torben Sonneck
AU - Michaela Barnikel
AU - Paola Arnold
AU - Gabriela Leuschner
AU - Amanda Tufman
AU - Jürgen Behr
PY - 2026
DA - 2026/11/03
JO - Journal of Clinical Medicine
DO - 10.3390/jcm15218574
UR - https://www.mdpi.com/2077-0383/15/21/8574
AB - Background/Objectives: Patients with pulmonary arterial hypertension (PAH) may display obstructive ventilatory defects outside of defined obstructive lung disease. However, its pathogenesis and prognostic relevance remain unknown. Similar data have emerged for patients with chronic thromboembolic pulmonary hypertension (CTEPH), but there is no description in a real-world European cohort. Methods: We performed a single-center, retrospective, cross-sectional study of patients with PAH (n = 46) and CTEPH (n = 45) at an academic PH center. Data were collected on pulmonary function tests, DLCO, right heart catheterization results, pre-existing diagnoses of asthma and chronic obstructive pulmonary disease (COPD), current medications, and adverse outcomes. Results: Patients with PAH and CTEPH showed a reduced FVC (81% for both groups), FEV1 (74 and 75%) and DLCO (54 and 53%). A previous diagnosis of COPD was documented in 11% of patients, and asthma in 11% of patients with PAH and 4.5% of patients with CTEPH respectively. Even after excluding patients with asthma or COPD, ventilatory obstruction was still present in at least 22% of patients and 16% were being treated with long-acting bronchodilators or inhaled corticosteroids. Of these patients, those who had adverse outcomes (active listing for lung transplantation, lung transplantation or death) had a significantly lower FVC (68 vs. 83%, p = 0.026), FEV1 (61 vs. 79%, p = 0.01) and MEF50 (38 vs. 59%, p = 0.021). All patients with adverse outcomes had a mean expiratory flow at 50% vital capacity (MEF50) < 60%, as opposed to 57% of those without an adverse event, however MEF50 did not predict adverse outcomes after adjusting for FVC. Conclusions: Ventilatory obstruction is common in patients with PAH and CTEPH even when excluding patients with asthma/COPD or a history of smoking. Symptomatic treatment with bronchodilators is common even without these comorbidities. Patients with adverse outcomes had lower FEV1, FVC and MEF50, but the functional relevance of the latter remains to be explored.
SN - 2077-0383
ER -