scholarly journals Palliative care in pulmonary arterial hypertension: an underutilised treatment

2018 ◽  
Vol 27 (150) ◽  
pp. 180069 ◽  
Author(s):  
Ghaleb Khirfan ◽  
Adriano R. Tonelli ◽  
Jennifer Ramsey ◽  
Sandeep Sahay
Keyword(s):  
Quality Of Life ◽  
Palliative Care ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Future Research ◽  
Number Of Patients ◽  
Pulmonary Arterial ◽  
The Impact

Pulmonary arterial hypertension (PAH) is a condition characterised by increased pulmonary vascular resistance which can lead to right heart failure and premature death. It imposes a significant burden on patients' lives, affecting their physical, emotional and social wellbeing. Pharmacological therapies are the mainstay of treatment; while they are not curative, they can alleviate patient suffering, improve quality of life and delay disease progression. Despite these therapies, disease progresses in a significant number of patients, who are faced with the debilitating symptoms of PAH and treatment adverse effects. Palliative care is focused on providing relief from symptoms caused by a chronic illness. Palliative care aims to improve the health-related quality of life for patients and families, and although it is deemed appropriate at any stage of disease, it is most helpful when explored early in the course of disease. Importantly, palliative care can be provided in concert with pharmacological treatment. Despite its potential benefits, palliative care is frequently underutilised. There is a paucity of clinical studies testing the impact of palliative care in PAH which prompted us to summarise the available evidence, recognise obstacles in its utilisation and identify areas for future research.

scholarly journals Impact of SARS-CoV-2-Pandemic on Mental Disorders and Quality of Life in Patients With Pulmonary Arterial Hypertension

2021 ◽  
Vol 12 ◽  
Author(s):  
Da Hee Park ◽  
Jan Fuge ◽  
Tanja Meltendorf ◽  
Kai G. Kahl ◽  
Manuel J. Richter ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Care ◽  
Anxiety And Depression ◽  
Pulmonary Arterial ◽  
Access To Medical Care ◽  
The Impact

Background/Objective: Covid-19 pandemic may affect mental health and quality of life (QoL) in patients with pulmonary arterial hypertension (PAH). We assessed changes in anxiety and depression, quality of life (QoL) and self-described impact of Covid-19 in patients with PAH during the Covid-19 pandemic.Methods: This study included 152 patients with PAH from two German referral centers. Anxiety and depression were assessed using the Hospital Anxiety and Depression Scale (HADS-A and HADS-D) at two different timepoints before and during the Covid-19 pandemic with a median of 232 days between baseline and follow-up. QoL was assessed using EQ-5D and emPHasis-10. Perceived impact of Covid-19 and related regulations and measures were assessed using a set of specific questions and statements.Results: More than two thirds of patients had an unsuspicious HADS-A and HADS-D. Median scores did not differ from baseline for both HADS-A and HADS-D (p = 0.202; p = 0.621). Overall, no significant changes in HADS-A or HADS-D categories from baseline to follow up were observed (p = 0.07; p = 0.13). QoL did not change between baseline and follow-up. The Covid-19 pandemic had little impact on access to medical care and established PAH therapy. Patients were in agreement with governmental measures and regulations and felt sufficiently safe.Conclusion: First waves of Covid-19 pandemic had little impact on anxiety, depression and QoL in patient with PAH. Established PAH therapy and access to medical care were not affected. Further studies on the impact of prolonged duration of the ongoing Covid-19 pandemic are needed.

scholarly journals Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension

2020 ◽  
Vol 9 (2) ◽  
pp. 410 ◽  
Author(s):  
Marianne Riou ◽  
Mégane Pizzimenti ◽  
Irina Enache ◽  
Anne Charloux ◽  
Mathieu Canuet ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Respiratory Muscle ◽  
Right Heart Failure ◽  
Exercise Intolerance ◽  
Research Areas ◽  
Pulmonary Arterial ◽  
Underlying Mechanisms

Pulmonary arterial hypertension (PAH) is a rare disease, which leads to the progressive loss and remodeling of the pulmonary vessels, right heart failure, and death. Different clinical presentations can be responsible for such a bad prognosis disease and the underlying mechanisms still need to be further examined. Importantly, skeletal and respiratory muscle abnormalities largely contribute to the decreased quality of life and exercise intolerance observed in patients with PAH. At the systemic level, impaired oxygen supply through reduced cardiac output and respiratory muscle dysfunctions, which potentially result in hypoxemia, is associated with altered muscles vascularization, inflammation, enhanced catabolic pathways, and impaired oxygen use through mitochondrial dysfunctions that are likely participate in PAH-related myopathy. Sharing new insights into the pathological mechanisms of PAH might help stimulate specific research areas, improving the treatment and quality of life of PAH patients. Indeed, many of these muscular impairments are reversible, strongly supporting the development of effective preventive and/or therapeutic approaches, including mitochondrial protection and exercise training.

scholarly journals Pulmonary arterial hypertension: guidelines and unmet clinical needs

Reumatismo ◽  
2021 ◽  
Vol 72 (4) ◽  
pp. 228-246
Author(s):  
D. Giuggioli ◽  
C. Bruni ◽  
F. Cacciapaglia ◽  
F. Dardi ◽  
A. De Cata ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Negative Impact ◽  
Right Heart Failure ◽  
European Respiratory Society ◽  
Right Heart ◽  
Screening Programs ◽  
Pulmonary Arterial

The term pulmonary arterial hypertension (PAH) identifies a heterogeneous group of diseases characterized by a progressive increase in pulmonary arterial resistance (PVR), which causes a significant burden in terms of quality of life, right heart failure and premature death. The pathogenesis of PAH is not completely clear: the remodeling of the small pulmonary vessels is crucial, causing an increase in the resistance of the pulmonary circle. Its diagnosis is based on cardiac catheterization of the right heart. According to the present hemodynamic definition of pulmonary hypertension (PH) proposed by the Guidelines of the European Society of Cardiology/European Respiratory Society (ESC-ERS), the mean pulmonary arterial pressure (mPAP) values are ≥25 mmHg. In case of PAH, apart from an mPAP value ≥25 mmHg, patients must have a >3 Wood units increase in PVR and normal pressure values of the left heart. PH is a pathophysiological condition observed in more than 40 different diseases, while PAH is a primary disease of the pulmonary bloodstream potentially treatable with specific drugs. PAH is a severe complication of systemic sclerosis (SSc) affecting about 10% of the patients. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. In fact, despite awareness of the negative impact of SSc-PAH on quality of life and survival, as well as on the severity of lung function, at the moment standardized and shared guidelines and/or screening programs for the diagnosis and the subsequent early treatment of PAH in SSc are not available. The aim of the present paper is to highlight the lights and shadows of SSc-PAH, unraveling the unmet clinical needs on this topic with a proposal of clinical-diagnostic and therapeutic guidelines.

scholarly journals EXPRESS: Association of daily physical activity with psychosocial aspects and functional capacity in patients with Pulmonary Arterial Hypertension: a cross-sectional study

2021 ◽  
pp. 204589402199995
Author(s):  
Layse Nakazato Lima ◽  
Felipe Mendes ◽  
Ilma Paschoal ◽  
Daniela Oliveira ◽  
Marcos Mello Moreira ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Physical Activity ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Capacity ◽  
Daily Physical Activity ◽  
Cross Sectional ◽  
Pulmonary Arterial ◽  
The Mean

Pulmonary arterial hypertension (PAH) impairs exercise tolerance and daily physical activity (PA). Aside from the hemodynamic limitations, physical, cognitive and emotional factors may play a relevant and as yet unexplored role. We investigated whether there is an association between the PA level and psychological disorders, health-related quality of life, and daily activities. We also searched for an association of the PA level with clinical factors and functional capacity. This was an analytical, cross-sectional, observational study conducted in a Brazilian University Hospital. Twenty stable PAH subjects wore an accelerometer for a week and completed an activity diary. They answered the quality of life questionnaire (SF-36), as well as the anxiety and depression scale (HADS), and the Manchester Respiratory Activities of Daily Living questionnaire (MRADL). Transthoracic echocardiography, the 6-Minute walk test (6MWT), the 1-minute sit-to-stand test (STST), and spirometry were performed. For statistical analysis we used Chi-square tests or Fisher's test as appropriate and the Mann-Whitney test to compare numerical values between two groups. The relationship between the parameters was assessed using the Spearman correlation test. The mean age was 44.3 years, 80% were women, 80% had idiopathic PAH, and 20% had connective tissue disease . The mean daily step count was 4,280 ± 2,351, and the mean activity time was 41.6 ± 19.3 minutes. The distance covered (6MWT) was 451.5 m, and the number of movements (1-STST) was 23.8. Thirty percent scored positive for anxiety, and 15% for depression (HADS). There was a significant correlation between accelerometer data and walking distance (6MWT), number of movements (1-STST), level of daily physical activity (MRADL), and depression symptoms. Our findings support the hypothesis that other aspects beyond physical and hemodynamic ones might impact the daily physical activity of patients with PAH.

Effect of Pulmonary Arterial Hypertension-Specific Therapies on Health-Related Quality of Life

CHEST Journal ◽  
2014 ◽  
Vol 146 (3) ◽  
pp. 686-708 ◽  
Author(s):  
Gilles Rival ◽  
Yves Lacasse ◽  
Sylvie Martin ◽  
Sébastien Bonnet ◽  
Steeve Provencher
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related ◽  
Pulmonary Arterial

scholarly journals A prescribed walking regimen plus arginine supplementation improves function and quality of life for patients with pulmonary arterial hypertension: a pilot study

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774396 ◽  
Author(s):  
Mary Beth Brown ◽  
Attie Kempf ◽  
Catherine M. Collins ◽  
Gary M. Long ◽  
Matthew Owens ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Current Evidence ◽  
Proof Of Concept ◽  
Concept Study ◽  
Sf 36 ◽  
Pulmonary Arterial ◽  
Arginine Supplementation

Current evidence suggests that exercise training is beneficial in pulmonary arterial hypertension (PAH). Unfortunately, the standard supervised, hospital-based programs limit patient accessibility to this important intervention. Our proof-of-concept study aimed to provide insight into the usefulness of a prescribed walking regimen along with arginine supplementation to improve outcomes for patients with PAH. Twelve PAH patients (all women) in New York Heart Association (NYHA) functional class (FC) II (n = 7) or III (n = 5) and in stable condition for ≥ 3 months were enrolled. Patients performed home- and fitness-center- based walking at 65–75% heart rate (HR) reserve for 45 min, six sessions/week for 12 weeks. Concomitant L-arginine supplementation (6000 mg/day) was provided to maximize beneficial endothelial training adaptations. Cardiopulmonary exercise testing, 6-min walk testing (6MWT), echocardiography, laboratory studies, and quality of life (QoL) survey (SF-36) were performed at baseline and 12 weeks. Eleven patients completed the study (72 session adherence rate = 96 ± 3%). Objective improvement was demonstrated by the 6MWT distance (increased by 40 ± 13 m, P = 0.01), VO2max (increased by 2 ± 0.7 mL/kg/min, P = 0.02), time-to-VO2max (increased by 2.5 ± 0.6 min, P = 0.001), VO2 at anaerobic threshold (increased by 1.3 ± 0.5 mL/kg/min, P = 0.04), HR recovery (reduced by 68 ± 23% in slope, P = 0.01), and SF-36 subscales of Physical Functioning and Energy/Fatigue (increased by 70 ± 34% and 74 ± 34%, respectively, P < 0.05). No adverse events occurred, and right ventricular function and brain natriuretic peptide levels remained stable, suggesting safety of the intervention. This proof-of-concept study indicates that a simple walking regimen with arginine supplementation is a safe and efficacious intervention for clinically stable PAH patients, with gains in objective function and QoL measures. Further investigation in a randomized controlled trial is warranted.

scholarly journals The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

2015 ◽  
Vol 46 (3) ◽  
pp. 832-842 ◽  
Author(s):  
Emmy Manders ◽  
Silvia Rain ◽  
Harm-Jan Bogaard ◽  
M. Louis Handoko ◽  
Ger J.M. Stienen ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Exercise Intolerance ◽  
Muscle Dysfunction ◽  
Striated Muscles ◽  
Pulmonary Arterial ◽  
The Right ◽  
The Impact

Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction.

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