ArticleAmerican journal of physiology. Lung cellular and molecular physiology2016
Reduced force of diaphragm muscle fibers in patients with chronic thromboembolic pulmonary hypertension.
Article in American journal of physiology. Lung cellular and molecular physiology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- Effectiveness and safety of inspiratory muscle training in patients with pulmonary hypertension: A systematic review and meta-analysis.Frontiers in cardiovascular medicine · 2022Pooled it
- Complementary multi-omics profiling of chronic thromboembolic pulmonary hypertension reveals immune cell alterations, epigenetic changes, and genetically supported candidate genes.Animal models and experimental medicine · 2026Article
- Physical exercise for pulmonary arterial hypertension diagnosis and therapy.International journal of cardiology. Congenital heart disease · 2025Review
- Exercise intolerance in pulmonary hypertension: robbing Peter to pay Paul.The Journal of physiology · 2025Article
- The impact of group II pulmonary hypertension on congestive heart failure patients admitted with ST elevation myocardial infarction, a nationwide study.Journal of thoracic disease · 2024Article
- Diaphragmatic ultrasound evaluation in acute heart failure: clinical and functional associations.Internal and emergency medicine · 2024Article
- Small molecule drugs to improve sarcomere function in those with acquired and inherited myopathies.American journal of physiology. Cell physiology · 2023Review
- Sex Differences in Chronic Thromboembolic Pulmonary Hypertension. Treatment Options over Time in a National Referral Center.Journal of clinical medicine · 2021Article
- Acute and chronic tirasemtiv treatment improves in vivo and in vitro muscle performance in actin-based nemaline myopathy mice.Human molecular genetics · 2021Article
- Pulmonary Vascular Disease and Cardiopulmonary Exercise Testing.Frontiers in physiology · 2020Review
- Muscle weakness in aAnnals of translational medicine · 2019Article
- Increasing quality of life in pulmonary arterial hypertension: is there a role for nutrition?Heart failure reviews · 2018Review
- Diaphragm function and weaning from mechanical ventilation: an ultrasound and phrenic nerve stimulation clinical study.Annals of intensive care · 2018Article
- Breathing: Motor Control of Diaphragm Muscle.Physiology (Bethesda, Md.) · 2018Review
- Inspiratory muscle weakness contributes to exertional dyspnea in chronic thromboembolic pulmonary hypertension.PloS one · 2018Article
- Exertional dyspnoea in pulmonary arterial hypertension.European respiratory review : an official journal of the European Respiratory Society · 2017Review
- Changes in muscle fiber contractility and extracellular matrix production during skeletal muscle hypertrophy.Journal of applied physiology (Bethesda, Md. : 1985) · 2017Article
- Inspiratory capacity is not altered in operable chronic thromboembolic pulmonary hypertension.Pulmonary circulationArticle
- Clinical and imaging outcomes after intermediate- or high-risk pulmonary embolus.Pulmonary circulationArticle
- New method for evaluating maximal respiratory pressures: Concurrent validity, test-retest, and inter-rater reliability.Brazilian journal of physical therapyArticle
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Authors and funding
12 authors at 3 institutions in 2 countries.
Funding
Abstract
Patients with pulmonary hypertension (PH) suffer from inspiratory muscle weakness. However, the pathophysiology of inspiratory muscle dysfunction in PH is unknown. We hypothesized that weakness of the diaphragm, the main inspiratory muscle, is an important contributor to inspiratory muscle dysfunction in PH patients. Our objective was to combine ex vivo diaphragm muscle fiber contractility measurements with measures of in vivo inspiratory muscle function in chronic thromboembolic pulmonary hypertension (CTEPH) patients. To assess diaphragm muscle contractility, function was studied in vivo by maximum inspiratory pressure (MIP) and ex vivo in diaphragm biopsies of the same CTEPH patients (N = 13) obtained during pulmonary endarterectomy. Patients undergoing elective lung surgery served as controls (N = 15). Muscle fiber cross-sectional area (CSA) was determined in cryosections and contractility in permeabilized muscle fibers. Diaphragm muscle fiber CSA was not significantly different between control and CTEPH patients in both slow-twitch and fast-twitch fibers. Maximal force-generating capacity was significantly lower in slow-twitch muscle fibers of CTEPH patients, whereas no difference was observed in fast-twitch muscle fibers. The maximal force of diaphragm muscle fibers correlated significantly with MIP. The calcium sensitivity of force generation was significantly reduced in fast-twitch muscle fibers of CTEPH patients, resulting in a ∼40% reduction of submaximal force generation. The fast skeletal troponin activator CK-2066260 (5 μM) restored submaximal force generation to levels exceeding those observed in control subjects. In conclusion, diaphragm muscle fiber contractility is hampered in CTEPH patients and contributes to the reduced function of the inspiratory muscles in CTEPH patients.
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