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

Manders, Emmy; Rain, Silvia; Bogaard, Harm Jan; Handoko, M. Louis; Stienen, G. J. M.; Vonk‐Noordegraaf, Anton; Ottenheijm, Coen A.C.; Man, Frances S. de

doi:10.1183/13993003.02052-2014

Cited by 40 publications

(32 citation statements)

References 83 publications

(122 reference statements)

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“…Skeletal muscle dysfunction and severe, chronic deconditioning may be an underestimated problem in PAH patients [12]. With respect to the Fick equation, skeletal muscle function during exercise relates to Ca-vO 2 , as remaining organ oxygen demand is assumed to be relatively constant during exercise, and an increase in oxygen extraction is mainly caused by increased skeletal muscle metabolism.…”

Section: Cardiovascular Response To Exercise In Pahmentioning

confidence: 99%

“…Unlike muscles, which may increase in strength/endurance with increased loading, there is mixed evidence of respiratory muscle weakness in pulmonary hypertension [12,29,31,32]. Although this does not lead to exercise limitation, it is conceivable, although unproven, that this may contribute to the sensation of dyspnoea.…”

Section: Respiratory Response To Exercise In Pahmentioning

confidence: 99%

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Exertional dyspnoea in pulmonary arterial hypertension

et al. 2017

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Dyspnoea is a principal presenting symptom in pulmonary arterial hypertension (PAH), and often the most distressing. The pathophysiology of PAH is relatively well understood, with the primary abnormality of pulmonary vascular disease resulting in a combination of impaired cardiac output on exercise and abnormal gas exchange, both contributing to increased ventilatory drive. However, increased ventilatory drive is not the sole explanation for the complex neurophysiological and neuropsychological symptom of dyspnoea, with other significant contributions from skeletal muscle reflexes, respiratory muscle function, and psychological and emotional status. In this review, we explore the physiological aspects of dyspnoea in PAH, both in terms of the central cardiopulmonary abnormalities of PAH and the wider, systemic impact of PAH, and how these interact with common comorbidities. Finally, we discuss its relationship with disease severity.

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Section: Cardiovascular Response To Exercise In Pahmentioning

confidence: 99%

Section: Respiratory Response To Exercise In Pahmentioning

confidence: 99%

Exertional dyspnoea in pulmonary arterial hypertension

et al. 2017

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“…Skeletal muscle abnormalities have been demonstrated in patients with pulmonary arterial hypertension PAH [79][80][81] . As in COPD, skeletal muscle weakness in PAH patients leads to increased dyspnoea, fatigue, poor exercise tolerance, and impaired quality of life [79][80][81] .…”

Section: Pulmonary Arterial Hypertensionmentioning

confidence: 99%

“…As in COPD, skeletal muscle weakness in PAH patients leads to increased dyspnoea, fatigue, poor exercise tolerance, and impaired quality of life [79][80][81] . Similarly, a switch to a less fatigue-resistant phenotype (lower proportions of slowtwitch fibres) and increased anaerobic metabolism have been demonstrated in the lower limb muscles of patients with PAH [82][83][84] .…”

Section: Pulmonary Arterial Hypertensionmentioning

confidence: 99%

Skeletal Muscle Dysfunction: From Chronic Respiratory Disease to Critically Ill Patients

Barreiro¹

2016

BRN

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Muscle dysfunction, defined as the loss of either strength or endurance properties of muscles, is a prominent comorbidity in chronic obstructive pulmonary disease (COPD) that leads to impairment of the patients' exercise capacity and quality of life. Chronic obstructive pulmonary disease skeletal muscle dysfunction may affect both respiratory and limb muscles. However, the latter are usually more severely affected. Importantly, survival and COPD mortality are negatively influenced by dysfunction of the lower limb muscles and poor muscle mass as measured by mid-thigh cross-sectional area. In the current review, we discuss the effects of skeletal muscle dysfunction and wasting on the outcome of COPD. Additionally, whether muscle dysfunction takes place in respiratory disorders other than COPD, such as cystic fibrosis, idiopathic pulmonary fibrosis, scoliosis, and pulmonary arterial hypertension along with critical illness, is also reviewed herein. Identification of skeletal muscle dysfunction should be included in the routine assessment of patients with chronic respiratory disorders and in critical care settings and should be considered as a relevant target for treatment. Corresponding author: Esther Barreiro, ebarreiro@imim.es

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“…Finally, it is important to point out that use of non-pharmacological interventions like participation in exercise training programs has been advocated for the patient with PAH as they have been shown to increase exercise capacity, pulmonary hemodynamics, and health-related quality of life 12 . This is relevant in light of recent studies that have shown atrophy and dysfunction of striated muscles associated with the respiratory and musculoskeletal systems 13 .…”

Section: Introductionmentioning

confidence: 97%

Recent advances in the management of pulmonary arterial hypertension

2016

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Over the past 20 years, there has been an explosion in the development of therapeutics to treat pulmonary arterial hypertension (PAH), a rare but life-threatening disorder associated with progressive elevation of pulmonary pressures and severe right heart failure. Recently, the field has seen the introduction of riociguat, a soluble guanylate cyclase stimulator, a new endothelin receptor antagonist (macitentan), and oral prostanoids (treprostinil and selexipag). Besides new drugs, there have been significant advances in defining the role of upfront combination therapy in treatment-naïve patients as well as proposed methods to deliver systemic prostanoids by use of implantable pumps. In this review, we will touch upon the most important developments in PAH therapeutics over the last three years and how these have changed the guidelines for the treatment of PAH. These exciting developments herald a new era in the treatment of PAH which will be punctuated by the use of more clinically relevant endpoints in clinical research trials and a novel treatment paradigm that may involve upfront double- or triple-combination therapy. We anticipate that the future will make use of these strategies to test the efficacy of upcoming new drugs that aspire to reduce disease progression and improve survival in patients afflicted with this devastating disease.

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

Cited by 40 publications

References 83 publications

Exertional dyspnoea in pulmonary arterial hypertension

Exertional dyspnoea in pulmonary arterial hypertension

Skeletal Muscle Dysfunction: From Chronic Respiratory Disease to Critically Ill Patients

Recent advances in the management of pulmonary arterial hypertension

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