Skeletal Muscle Abnormalities in Pulmonary Arterial Hypertension

Breda, Ana Paula; Albuquerque, André Luís Pereira de; Jardim, Carlos; Morinaga, Luciana Tamie Kato; Suesada, Milena; Fernandes, Constantino José; Dias, Bruno; Lourenço, Rafael Burgomeister; Salge, João Marcos; Souza, Rogério

doi:10.1371/journal.pone.0114101

Cited by 45 publications

(47 citation statements)

References 40 publications

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“…The present estimation seems accurate, as we used a recommended GFR formula to evaluate renal function [21]. However, all GFR estimation methods that rely on creatinine assays may underestimate the true prevalence of CKD in sarcopenic patients [29]. Porto-pulmonary hypertension patients may suffer from such an estimation bias as eGFR is frequently overestimated in cirrhotic patients.…”

Section: Discussionmentioning

confidence: 99%

Presence of Kidney Disease as an Outcome Predictor in Patients with Pulmonary Arterial Hypertension

et al. 2018

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Background: Pulmonary arterial hypertension (PAH) may lead to right heart failure and subsequently alter glomerular filtration rates (GFR). Chronic kidney disease (CKD, GFR <60 mL/min/1.73 m²) may also adversely affect PAH prognosis. This study aimed to assess how right heart hemodynamics was associated with reduced estimated GFR (eGFR) and the association of CKD with survival in PAH patients. Methods: In a prospective PAH cohort (2003–2012), invasive hemodynamics and eGFR were collected at diagnosis (179 patients) and during follow-up (159 patients). The prevalence of CKD was assessed at PAH diagnosis. Variables, including hemodynamics, associated with reduced eGFR at diagnosis and during follow-up were tested in multivariate analysis. The association of CKD with survival was evaluated using a multivariate Cox regression model. Results: At diagnosis, mean age was 60.4 ± 16.5 years, mean pulmonary arterial pressure was 43 ± 12 mm Hg, and eGFR was 74.4 ± 26.4 mL/min/1.73 m². CKD was observed in 52 incident patients (29%). Independent determinants of reduced eGFR at diagnosis were age, systemic hypertension, and decreased cardiac index. Independent determinants of reduced eGFR during follow-up were age, female gender, PAH etiology, systemic hypertension, decreased cardiac index, and increased right atrial pressure. Age ≥60 years, female gender, NYHA 4, and CKD at diagnosis were independently associated with decreased survival. The adjusted hazards ratio for death associated with CKD was 1.81 (95% confidence interval [1.01–3.25]). Conclusion: CKD is frequent at PAH diagnosis and is independently associated with increased mortality. Right heart failure may induce renal hypoperfusion and congestion, and is associated with eGFR decrease.

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Section: Discussionmentioning

confidence: 99%

Presence of Kidney Disease as an Outcome Predictor in Patients with Pulmonary Arterial Hypertension

et al. 2018

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“…Furthermore, patients with PHTN have respiratory muscle dysfunction, a factor that contributes to the pre‐existing hypoventilatory state in patients with DP . Other important contributing factors are atrophy of the diaphragm and a significant reduction in the forced generating capacity of the diaphragm in patients with PHTN . We found an association between the MLIS and the risk of requiring a DPL procedure.…”

Section: Discussionmentioning

confidence: 71%

Predictors for plication performance following diaphragmatic paralysis in children

Goldberg

Krauthammer

Ashkenazi

et al. 2019

Pediatric Pulmonology

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Introduction and Objectives Diaphragmatic paralysis (DP) in children can result from various etiologies. Guidelines for patient selection for diaphragmatic plication (DPL) are lacking. Our objectives were to describe the etiologies of DP and to determine the risk factors and predictors for DPL in the pediatric population. Methods Retrospective data were retrieved from departmental databases on patients with DP from the pediatric, cardiac, and neonatal intensive care departments of Safra Children's Hospital from 2010 to 2017. Results DP was diagnosed in 88 patients, 29 with noncardiac surgery‐related etiologies, for example, congenital, surgery, trauma, and shock and 59 with cardiac surgery‐related etiologies. In total, 27 (31%) patients underwent DPL, and they had significant comorbidities involving respiratory, central nervous, and cardiovascular systems, higher lung injury scores, and lower weight compared with the patients who did not undergo DPL (P = .002, P = .002, P < .001, P = .012, and P = .013, respectively). A multivariate regression model revealed significant independent predictors for DPL, including morbidities of central nervous (odds ratio [OR = 9.651, P = .005), respiratory (OR = 4.875, P = .039), and cardiovascular systems (OR = 23.938, P = .001). Conclusions Etiologies of DP are very diverse in the pediatric population. Comorbidities of respiratory, central nervous, and cardiovascular systems are risk factors for plication requirement in respiratory support‐dependent pediatric patients with DP. Early DPL should be considered in these patients.

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“…Maximal volitional and nonvolitional strength of both the inspiratory and quadriceps muscles are reduced in PAH patients, a reduction that correlates with exercise capacity [7,9,44,45]. Maximal muscle strength does not depend directly on oxygen supply, indicating that the intrinsic capacity of the skeletal muscle to generate force is impaired.…”

Section: Local Factorsmentioning

confidence: 99%

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

et al. 2015

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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. @ERSpublications RV, LV, diaphragm and peripheral muscle dysfunction contributes to reduced quality of life in PAH patients

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Skeletal Muscle Abnormalities in Pulmonary Arterial Hypertension

Cited by 45 publications

References 40 publications

Presence of Kidney Disease as an Outcome Predictor in Patients with Pulmonary Arterial Hypertension

Presence of Kidney Disease as an Outcome Predictor in Patients with Pulmonary Arterial Hypertension

Predictors for plication performance following diaphragmatic paralysis in children

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

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