scholarly journals Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial

2020 ◽  
Author(s):  
Ekkehard Grünig ◽  
Alison MacKenzie ◽  
Andrew J Peacock ◽  
Christina A Eichstaedt ◽  
Nicola Benjamin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Exercise Training ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
European Countries ◽  
Walking Distance ◽  
Control Group ◽  
Randomized Controlled ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Abstract Aims This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods and results For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across 10 European countries. Out of 129 enrolled patients, 116 patients (58 vs. 58 randomized into a training or usual care control group) on disease-targeted medication completed the study [85 female; mean age 53.6 ± 12.5 years; mean pulmonary arterial pressure 46.6 ± 15.1 mmHg; World Health Organization (WHO) functional class II 53%, III 46%; PAH n = 98; CTEPH n = 18]. Patients of the training group performed a standardized in-hospital rehabilitation with mean duration of 25 days [95% confidence interval (CI) 17–33 days], which was continued at home. The primary endpoint, change of 6-min walking distance, significantly improved by 34.1 ± 8.3 m in the training compared with the control group (95% CI, 18–51 m; P < 0.0001). Exercise training was feasible, safe, and well-tolerated. Secondary endpoints showed improvements in quality of life (short-form health survey 36 mental health 7.3 ± 2.5, P = 0.004), WHO-functional class (training vs. control: improvement 9:1, worsening 4:3; χ2  P = 0.027) and peak oxygen consumption (0.9 ± 0.5 mL/min/kg, P = 0.048) compared with the control group. Conclusion This is the first multicentre and so far the largest randomized, controlled study on feasibility, safety, and efficacy of exercise training as add-on to medical therapy in PAH and CTEPH. Within this study, a standardized specialized training programme with in-hospital start was successfully established in 10 European countries.

Effect of Supervised Training Therapy on Pulmonary Arterial Compliance and Stroke Volume in Severe Pulmonary Arterial Hypertension and Inoperable or Persistent Chronic Thromboembolic Pulmonary Hypertension

Respiration ◽  
2021 ◽  
pp. 1-10
Author(s):  
Christian Nagel ◽  
Nicola Benjamin ◽  
Benjamin Egenlauf ◽  
Christina A. Eichstaedt ◽  
Christine Fischer ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Exercise Training ◽  
Stroke Volume ◽  
Arterial Compliance ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Training Group ◽  
Control Group ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

<b><i>Background:</i></b> Pulmonary arterial compliance (PAC) is a prognostic parameter in pulmonary arterial hypertension (PAH) reflecting the elasticity of the pulmonary vessels. <b><i>Objectives:</i></b> The objective of this post hoc analysis of a prospective randomized controlled trial (RCT) was to assess the effect of exercise training on PAC and stroke volume (SV) in patients with PAH and persistent/inoperable chronic thromboembolic pulmonary hypertension (CTEPH). <b><i>Method:</i></b> From the previous RCT, 43 out of 87 patients with severe PAH (<i>n</i> = 29) and CTEPH (<i>n</i> = 14) had complete haemodynamic examinations at baseline and after 15 weeks by right heart catheterization and were analysed (53% female, 79% World Health Organization functional class III/IV, 58% combination therapy, 42% on supplemental oxygen therapy, training group <i>n</i> = 24, and control group <i>n</i> = 19). Medication remained unchanged for all patients. <b><i>Results:</i></b> Low-dose exercise training at 4–7 days/week significantly improved PAC (training group 0.33 ± 0.65 mL/mm Hg vs. control group −0.06 ± 1.10 mL/mm Hg; mean difference 0.39 mL/mm Hg, 95% confidence interval [CI] 0.15–0.94 mL/mm Hg; <i>p</i> = 0.004) and SV (training group 9.9 ± 13.4 mL/min vs. control group −4.2 ± 11.0 mL/min; mean difference 14.2 mL, 95% CI 6.5–21.8 mL; <i>p</i> &#x3c; 0.001) in the training versus control group. Furthermore, exercise training significantly improved cardiac output and pulmonary vascular resistance at rest, peak oxygen consumption, and oxygen pulse. <b><i>Conclusions:</i></b> Our findings suggest that supervised exercise training may improve right ventricular function and PAC at the same time. Further prospective studies are needed to evaluate these findings.

scholarly journals Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

2021 ◽  
Vol 10 (19) ◽  
pp. 4547
Author(s):  
Eftychia Demerouti ◽  
Panagiotis Karyofyllis ◽  
Vassilios Voudris ◽  
Maria Boutsikou ◽  
George Anastasiadis ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
World Health ◽  
Real World Data ◽  
Significant Difference ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
Pulmonary Arterial ◽  
Health Organization

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m2, respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33).

scholarly journals Evaluation of inflammatory and coagulation markers in patients with idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension with comorbid hypercholesterolemia

2019 ◽  
Vol 29 (2) ◽  
pp. 175-183
Author(s):  
Irina N. Taran ◽  
Ol’ga A. Arkhipova ◽  
Valeriy P. Masenko ◽  
Anatoliy B. Dobrovol’skiy ◽  
Tamila V. Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Control Group ◽  
Total Blood ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
D Dimer

The aim of the study was to assess inflammatory and coagulation disorders in patients with idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in dependence on dyslipidemia. Methods. The study involved 22 patients with IPAH and 6 patients with inoperable CTEPH complicated by hypercholesterolemia (total cholesterol (TC) level > 5.8 mmol/L). Healthy volunteers with normal TC level (n = 20) were included in a control group. We measured serum cytokine concentrations, such as interleukin (IL)-2, IL-4, IL-5, IL-6, IL-10, IL-12, and interferon (INF)-γ, using a multiplex fluorescent analyzer GEN-PROBE (Luminex 200) with Bio-Plex Precision Pro set. Von Willebrand factor was measured using an immunoturbidimetric assay with STA-Liatest VWF: Ag set. D-dimer, fibrinogen, C-reactive protein, and the total blood cell count were also measured. Results. CTERH patients had lower IL-2 level and more prominent signs of chronic heart failure (CHF) compared to patients with IPAH. Serum levels of IL-10, IL-4, and IL-12 were significantly related to syncope frequency, INF-γ level and cardiac index (CI). High density lipoprotein (HDL) level was inversely related to mean pulmonary arterial pressure (mPAP) in IPAH/CTEPH patients with TC > 6.5 mmol/L. Decreased thrombocyte count and decreased thrombocrit were associated with increased mPAP and mean right atrium (RA) pressure in patients with dilated right pulmonary artery. Increased D-dimer level was associated with enlarged RA area, enlarged right ventricle size, tricuspid regurgitation and decreased CI. Conclusion. Serum concentrations of the inflammatory and coagulation markers, excepting LI-2, did no differ significantly in patients with IPAH and CTEPH. Dyslipidemia was associated with activation of inflammation. Further studies are needed to estimate a predictive value of decreased HDL in patients with IPAH and CTEPH.

scholarly journals Phosphodiesterase type 5 inhibitor to riociguat transition is associated with hemodynamic and symptomatic improvement in pulmonary hypertension

2017 ◽  
Vol 7 (2) ◽  
pp. 539-542 ◽  
Author(s):  
Ryan Davey ◽  
Raymond L. Benza ◽  
Srinivas Murali ◽  
Amresh Raina
Keyword(s):  
Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Symptomatic Improvement ◽  
Functional Class ◽  
Phosphodiesterase Type 5 Inhibitor ◽  
Thromboembolic Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase Stimulator ◽  
Pulmonary Arterial ◽  
Phosphodiesterase Type

Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. We studied the clinical and hemodynamics effects of transitioning 12 pulmonary hypertension patients from Phosphodiesterase type 5 inhibitor (PDE5i) to riociguat, and demonstrated a significant increase in cardiac index, fall in pulmonary vascular resistance, and improvement in functional class with this switch. Switch from PDE5i to riociguat appeared to be safe and fairly well tolerated in most patients.

scholarly journals Decreased time constant of the pulmonary circulation in chronic thromboembolic pulmonary hypertension

2013 ◽  
Vol 305 (2) ◽  
pp. H259-H264 ◽  
Author(s):  
Robert V. MacKenzie Ross ◽  
Mark R. Toshner ◽  
Elaine Soon ◽  
Robert Naeije ◽  
Joanna Pepke-Zaba
Keyword(s):  
Pulmonary Hypertension ◽  
Time Constant ◽  
Pulmonary Circulation ◽  
Wave Reflection ◽  
Arterial Compliance ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Left Heart Failure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Rc Time Constant

This study analyzed the relationship between pulmonary vascular resistance (PVR) and pulmonary arterial compliance ( Ca) in patients with idiopathic pulmonary arterial hypertension (IPAH) and proximal chronic thromboembolic pulmonary hypertension (CTEPH). It has recently been shown that the time constant of the pulmonary circulation (RC time constant), or PVR × Ca, remains unaltered in various forms and severities of pulmonary hypertension, with the exception of left heart failure. We reasoned that increased wave reflection in proximal CTEPH would be another cause of the decreased RC time constant. We conducted a retrospective analysis of invasive pulmonary hemodynamic measurements in IPAH ( n = 78), proximal CTEPH ( n = 91) before (pre) and after (post) pulmonary endarterectomy (PEA), and distal CTEPH ( n = 53). Proximal CTEPH was defined by a postoperative mean pulmonary artery pressure (PAP) of ≤25 mmHg. Outcome measures were the RC time constant, PVR, Ca, and relationship between systolic and mean PAPs. The RC time constant for pre-PEA CTEPH was 0.49 ± 0.11 s compared with post-PEA-CTEPH (0.37 ± 0.11 s, P < 0.0001), IPAH (0.63 ± 0.14 s, P < 0.001), and distal CTEPH (0.55 ± 0.12 s, P < 0.05). A shorter RC time constant was associated with a disproportionate decrease in systolic PAP with respect to mean PAP. We concluded that the pulmonary RC time constant is decreased in proximal CTEPH compared with IPAH, pre- and post-PEA, which may be explained by increased wave reflection but also, importantly, by persistent structural changes after the removal of proximal obstructions. A reduced RC time constant in CTEPH is in accord with a wider pulse pressure and hence greater right ventricular work for a given mean PAP.

scholarly journals EXPRESS: What’s New in Pulmonary Hypertension Clinical Research: Lessons from the Best Abstracts at the 2020 American Thoracic Society International Conference

2021 ◽  
pp. 204589402110407
Author(s):  
Andrew J Sweatt ◽  
Raju Reddy ◽  
Farbod Rahaghi ◽  
Nadine Al-Naamani
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Research ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
American Thoracic Society ◽  
Future Directions ◽  
International Conference ◽  
Metabolic Dysregulation ◽  
Current State ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

In this conference paper, we review the 2020 American Thoracic Society (ATS) International Conference session titled, “What’s New in Pulmonary Hypertension Clinical Research: Lessons from the Best Abstracts”. This virtual mini-symposium took place on October 21, 2020, in lieu of the annual in-person ATS International Conference which was cancelled due to the COVID-19 pandemic. Seven clinical research abstracts were selected for presentation in the session, which encompassed five major themes: (1) standardizing diagnosis and management of pulmonary hypertension, (2) improving risk assessment in pulmonary arterial hypertension, (3) evaluating biomarkers of disease activity, (4) understanding metabolic dysregulation across the spectrum of pulmonary hypertension, and (5) advancing knowledge in chronic thromboembolic pulmonary hypertension. Focusing on these five thematic contexts we review the current state of knowledge, summarize presented research abstracts, appraise their significance and limitations, and then discuss relevant future directions in pulmonary hypertension clinical research.

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