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Pulmonary arterial hypertension: a progressive 

Pulmonary arterial hypertension: a progressive 

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Pulmonary arterial hypertension (PAH) is characterized by a continuous increase in precapillary pulmonary vascular resistance (PVR) with a progressive reduction of cardiac output (CO). Similar to what occurs in left heart failure (HF), this represents the initial phase of a syndrome characterized by the progressive development of dyspnea and fatigu...

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... is defined by a resting mean pulmonary artery pressure (PAP) ≥25 mmHg, a wedge pressure <15 mmHg and a PVR >3 Wood Units, all measured by right heart catheterization, in the absence of other causes of pre-capillary pulmonary hypertension (PH), namely lung diseases, chronic pulmonary thromboembolism or other rare diseases (1). The disproportionate afterload the right ventricle has to face is at the origin of its failure, followed by the reduction of cardiac output (CO), at first during exercise and then even at rest ( Figure 1) (2). Usually, the early stages of PAH are free from symptoms, as the right heart initially manages to maintain an adequate CO. ...
Context 2
... of these 16 studies exploited an association of 5,8,10,11,13,14,49). ...
Context 3
... rapid deterioration of RV performance with its subsequent compromised hemodynamic effect, the occurrence of arrhythmias and syncope have always been considered the major risks of an exercise program in severe PAH (2,41,76). ...

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... It may help to identify areas of impairment and tailor interventions to improve HRQoL. Interventions such as structured and supervised exercise training (endurance and strength training) have been shown to be beneficial for patients with PH as they improve exercise capacity, muscle function, pulmonary circulation and QoL [43,44]. Subjective physical exertion, measured with the BDS, a peripheral oxygen saturation below 85% and other safety cut-offs (as outlined by the ERS statement [45]) provide a safe way to monitor exercise training in PH patients. ...
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Introduction Patients with pulmonary hypertension (PH) have an impaired functional capacity and poor health-related quality of life (HRQoL). The one-minute sit-to-stand test (1-min STST) can be used for the assessment of functional capacity. Aims Our aim was to evaluate the 1-min STST performance and its association with patient-reported HRQoL in patients with PH. Methods We prospectively assessed functional capacity in 98 PH patients (mean age 66 ± 15 years, 55% female) using the 1-min STST. Patients had to stand up and sit down from a chair as many times as possible within one minute. Patients’ HRQoL was evaluated with the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire, which consists of the three subcategories symptoms, activities and quality of life (QoL). Results We observed a significant correlation of the 1-min STST performance with all HRQoL subcategories assessed with the CAMPHOR questionnaire: A lower number of 1-min STST repetitions correlated with more symptoms (rs = -.398, p < .001), worse functioning (rs = -.551, p < .001) and a decreased QoL (rs = -.407, p < .001). Furthermore, in the multivariable linear regression analysis, adjusted for age, sex, body mass index (BMI) and mean pulmonary artery pressure (mPAP), lower 1-min STST performance was an independent predictor for worse symptoms (est. β = -0.112, p = .003), activities (est. β = -0.198, p < .001) and QoL (est. β = -0.130, p < .001) assessed with the CAMPHOR questionnaire. Conclusion Our results indicate that regardless of age, sex, BMI and mPAP the 1-min STST performance is associated with all CAMPHOR HRQoL subcategories in patients with PH. Therefore, the 1-min STST performance might be a new option to assess functional capacity correlated to HRQoL in patients with PH.
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... Pulmonary arterial hypertension (PAH) is a rare, progressive disorder occurring 3-5 times more frequently in females (mostly between 30 and 60 yrs) than in males and characterized by an increased pulmonary vascular resistance (PVR) leading to right ventricular (RV) failure [1,2]. ...
... This is in contrast with data obtained in other cardiac conditions, reinforcing the fact that the PAH patients have peculiar aspects requiring specific therapeutic approaches, including training and rehabilitation programs. In fact, even in PAH patients with a high impairment of functional capacity, specific training has shown some benefits [1]. ...
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... In turn, RT practice improved this parameter in animals with PAH. The practice of physical exercise has already been associated with cardiac parameter improvement in animals with PAH, especially with aerobic exercises (Dalla Vecchia & Bussotti, 2018;Naci et al., 2019;Soares et al., 2019). Moreover, combined exercise interventions, including aerobic, resistance, and specific inspiratory muscle training, proved safe for PAH patients and led to significant improvements in muscle power, exercise capacity, and survival (González-Saiz et al., 2017;Zhang & Xu, 2020;Yan et al., 2021). ...
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... A total of 28 articles were included in this updated review of which nine were obtained from the above-mentioned search strategy, 17 were from the previous review, 6 and two 15,16 were obtained by back-referencing previous reviews 9, [10][11][12] (see Supplemental Digital Content 2, available at: http:// links.lww.com/JCRP/A436). The 28 studies consisted of 11 RCT 14-24 and 17 non-RCT. ...
... Pulmonary hypertension impacts activities of daily life, working abilities, psychosocial health, and overall HRQoL. 10,11 This review found that exercise training had no significant effect on overall HRQoL, but improved the physical and mental subcomponents. This finding is similar to the other reviews, which have analyzed subcomponents of the SF-36 individually. ...
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Purpose: Given that previous reviews on exercise training in pulmonary hypertension (PH) were largely based on a small number of randomized controlled trials (RCT), their conclusions are subject to bias. This review sought to identify the impact of exercise training on functional capacity and health-related quality of life (HRQoL) in PH using advanced statistical approaches such as meta-analysis by stratification according to study design. Review methods: Five databases were searched from January 2015 to April 2020 to update a previous review. Included articles had data extracted, risk of bias (ROB) assessed, and quality rating performed. Data were analyzed using meta-analysis with a random-effects model for 6-min walk test (6MWT) distance and HRQoL. Heterogeneity was explored using stratified meta-analysis, within patient correlation and meta-regression. Results: A total of 28 studies (11 RCT, 12 pre-/post-studies, 2 two-group non-RCT, and three case series) consisting of 1264 patients were included. Meta-analysis of six RCT demonstrated an improved 6MWT distance by 49.5 m (95% CI, 27.2-71.8: I2 = 73%; 254 participants; low-moderate ROB) with a low correlation coefficient of 0.34, while the 12 pre-/post-non-RCT showed an improvement of 68.69 m (95% CI, 50.50-86.69: I2 = 36%; 784 participants; high ROB) along with improvements in V˙ o2peak (weighted mean difference [WMD] = 3.03 mL/kg/min, 95% CI, 2.17-3.90: I2 = 0%, P = .82), and HRQoL (WMD = 2.74: 95% CI, -0.82 to 6.30). Metaregression showed that the benefit of exercise on 6MWT distance did not significantly vary across the trial study characteristics. Conclusion: This updated review identified an additional body of evidence supporting the efficacy of exercise training on 6MWT distance and HRQoL in stable PH patients. These benefits appeared to be consistent across models of delivery.
... Exercise exercises combined with pulmonary hypertension-specific drug therapy has shown beneficial effects in patients with CTEPH. (14) A randomized controlled trial study showed a significant increase in the total score of the physical and mental components scale after 15 weeks of exercise training compared to the control group. Exercise training also affected the patient's perception of fatigue as assessed by the Fatigue Severity Scale, the severity of fatigue was significantly reduced after 10 weeks of intervention compared to the control group. ...
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... Generally, PAH patients exhibit asthenia, fatigue, dyspnea, and poor scores of effort tolerance and quality of life. 6,7 Physical exercise training is a potential non-pharmacological tool to be used as a therapeutic option for cardiovascular diseases and complications. 1,7 Several physical training protocols have been used as promissory interventions in PAH experiments. ...
... 6,7 Physical exercise training is a potential non-pharmacological tool to be used as a therapeutic option for cardiovascular diseases and complications. 1,7 Several physical training protocols have been used as promissory interventions in PAH experiments. Continuous aerobic exercise protocols promoted beneficial effects in the right ventricle and pulmonary artery remodeling. ...
... It was reported that patients with PH generally had a worse prognosis than patients with advanced disease [2]. Pharmacotherapy for PH has been established for years to improve the prognosis of patients with PH; yet, the impact of pharmacotherapy remains unsatisfactory [3]. There are three wellknown pathways for PH pathophysiology and drugs targeting this pathway that has been well-established in clinical practice, such as PDE-5 inhibitors, prostanoid, and endothelin receptor antagonist. ...
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Patients with pulmonary hypertension (PH) continue to develop significant exertional symptoms and reduced quality of life despite receiving pharmacological therapy. Guidelines highlighted the importance of rehabilitation as part of management in PH. However, the cardiac rehabilitation program is still underused. We present a case of functional capacity improvement in patients with surgically corrected secundum atrial septal defect (ASD) with PH who underwent cardiac rehabilitation program. A 30-year-old female with a previous history of surgically corrected ASD went to our rehabilitation program in our hospital. She had a 3-weeks program of rehabilitation, and there were improvements in symptoms, clinical parameters, and quality of life after completion of the cardiac rehabilitation program. Rehabilitation can be an adjuvant for pharmacological therapy for PH that has been proven to improve the quality of life in patients with PH
... Confirming diagnosis of PH is done via right heart catheterization as patients have ≥ 25 mmHg resting mean pulmonary artery pressure (PAP) and < 15 mmHg pulmonary artery wedge pressure (PAWP) with pulmonary vascular resistance (PVR) > 3 Wood Units (WU) 1 Pulmonary rehabilitation is designed to improve physical and psychological condition of patients through comprehensive intervention based on patient-tailored therapies including exercise education, training, and behavior changes. 2 ...
... A randomized controlled comparative study among 30 patients with PAH, based on clinical, radiological and right heart catheter data in Kobry Al-Kobba military Chest hospital randomized equally into two groups. Group (1) included 15 patients recruited in 12-weeks exercise based pulmonary rehabilitation program while receiving medications and Group (2) included 15 patients only received medical treatment. ...
... The European Respiratory Society/European Cardiology Society (ERS/ESC) guidelines published in 2015 recommend the implementation of monitored individualized exercise training as adjuvant therapy for stable PAH patients on optimal medical treatment [8]. Since then, data regarding the effect of physical activity on disease progression and prognosis have been published [9][10][11][12][13][14][15][16]. Regrettably, a clinically useful definition of exercise for pulmonary hypertension is still not available [17], and diversity in training forms and models highlights the necessity for more precise analysis. ...
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Abstract Background Rehabilitation plays an important role in the management of patients with pulmonary arterial hypertension (PAH) and current guidelines recommend implementation of a monitored individualized exercise training program as adjuvant therapy for stable PAH patients on optimal medical treatment. An optimal rehabilitation model for this group of patients has not yet been established. This randomized prospective study assessed the effectiveness and safety of a 6-month home-based caregiver-supervised rehabilitation program among patients with pulmonary arterial hypertension. Methods A total of 39 patients with PAH were divided into two groups: intervention group (16 patients), subjected to a 6-month home-based physical training and respiratory rehabilitation program adapted to the clinical status of participants, and control group (23 patients) who did not perform physical training. The 6-min walk test (6MWT), measurement of respiratory muscle strength, quality of life assessment (SF-36, Fatigue Severity Scale – FSS) were performed before study commencement, and after 6 and 12 months. Adherence to exercise protocol and occurrence of adverse events were also assessed. Results Physical training significantly improved 6MWT distance (by 71.38 ± 83.4 m after 6 months (p = 0.004), which remained increased after 12 months (p = 0.043), and respiratory muscle strength after 6 and 12 months (p