Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials

2008 ◽  
Vol 67 (6) ◽  
pp. 808-814 ◽  
Author(s):  
J Avouac ◽  
J Wipff ◽  
A Kahan ◽  
Y Allanore
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Randomised Controlled Trials ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Pulmonary Arterial ◽  
Randomised Controlled

Safety and effect of sildenafil on treating paediatric pulmonary arterial hypertension: a meta-analysis on the randomised controlled trials

2020 ◽  
Vol 30 (12) ◽  
pp. 1882-1889
Author(s):  
Qingyou Zhang ◽  
Bowen Xu ◽  
Jichen Lv ◽  
Zhijian Wang ◽  
Junbao Du
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Randomised Controlled Trials ◽  
Meta Analysis ◽  
Statistical Significance ◽  
Hypertensive Crisis ◽  
Control Group ◽  
Controlled Trials ◽  
Pulmonary Arterial ◽  
Randomised Controlled

AbstractBackground:Efficacy of sildenafil in treating paediatric pulmonary arterial hypertension is controversial. This systematic review aimed to explore the safety and effect of sildenafil on treating paediatric pulmonary arterial hypertension (PAH) through meta-analysis.Methods and results:In this study, the electronic databases, including the Cochran Library database, EMBASE, and MEDLINE were systemically retrieved to identify the related randomised controlled trials (RCTs). Two reviewers had independently completed study selection, data collection, and assessment of the bias risk. Amongst 938 articles researched according to our retrieval strategy, 15 papers that involved 673 cases had been screened. Relative to control group, the sildenafil group had markedly reduced mortality (RR = 0.25, 95% CI: 0.12–0.51; p < 0.0001), but difference within the mortality was not statistically significant between high- and low-dose sildenafil groups (p = 0.152). Nonetheless, difference of the mean pulmonary arterial pressure between sildenafil as well as control group was of no statistical significance. Differences in the length of hospital stay and the incidences of pulmonary hypertensive crisis between children with PAH and controls were of no statistical significance. However, the summary estimate favoured that sildenafil reduced the duration of mechanical ventilation time, as well as the length of ICU stay and inotropic support.Conclusions:Sildenafil therapy reduces the mortality of PAH patients, but its effects on the haemodynamic outcomes and other clinical outcomes are still unclear.

scholarly journals Meta-Analysis of Randomized Controlled Trials on Treatment of Pulmonary Arterial Hypertension

2010 ◽  
Vol 74 (7) ◽  
pp. 1458-1464 ◽  
Author(s):  
Bing He ◽  
Fengwen Zhang ◽  
Xueying Li ◽  
Chaoshu Tang ◽  
Guosheng Lin ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Randomized Controlled Trials ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Randomized Controlled ◽  
Pulmonary Arterial

scholarly journals Nailfold Capillaroscopy in Systemic Sclerosis Patients with and without Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 10 (7) ◽  
pp. 1528
Author(s):  
Ioanna Minopoulou ◽  
Marieta Theodorakopoulou ◽  
Afroditi Boutou ◽  
Alexandra Arvanitaki ◽  
Georgia Pitsiou ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Meta Analysis ◽  
Capillary Density ◽  
Right Heart Catheterization ◽  
Nailfold Capillaroscopy ◽  
Cochrane Library ◽  
Heart Catheterization ◽  
Pulmonary Arterial

Systemic sclerosis (SSc)-related pulmonary arterial hypertension (SSc-PAH) is a leading cause of mortality in SSc. The extent of peripheral microvasculopathy assessed through nailfold capillaroscopy might correlate with the presence of PAH in SSc patients. We searched the PubMed, Cochrane Library, Scopus, and Web of Science databases and performed a random effects meta-analysis of observational studies comparing nailfold capillaroscopic alterations in SSc-PAH versus SSc-noPAH patients. Weighted mean differences (WMD) with the corresponding confidence intervals (CIs) were estimated. The quality of the included studies was evaluated using a modified Newcastle–Ottawa scale. Seven studies with 101 SSc-PAH and 277 SSc-noPAH participants were included. Capillary density was marginally reduced in the SSc-PAH group (WMD: −1.0, 95% CI: −2.0 to 0.0, I2 = 86%). This effect was strengthened once PAH diagnosis was confirmed by right heart catheterization (WMD: −1.2, 95% CI: −2.3 to −0.1, I2 = 85%). An increase in capillary loop width was observed in SSc-PAH compared to SSc-noPAH patients (WMD: 10.9, 95% CI: 2.5 to 19.4, I2 = 78%). Furthermore, SSc-PAH patients had a 7.3 times higher likelihood of active or late scleroderma pattern (95% CI: 3.0 to 18.0, I2 = 4%). SSc-PAH patients presented with worse nailfold capillaroscopic findings compared to SSc-noPAH patients.

Abstract 16011: An Outcomes Assessment of Vasodilator Therapy in Pulmonary Arterial Hypertension Through Meta-Analysis of 31 Randomized Controlled Trials

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
John J Ryan ◽  
Saurav Chatterjee ◽  
Nathan Hatton ◽  
Neel Patel ◽  
Josef Stehlik ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Randomized Controlled Trials ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Mortality Benefit ◽  
Randomized Controlled ◽  
Pulmonary Vasodilators ◽  
Pulmonary Arterial

Introduction: Pulmonary Arterial Hypertension (PAH) is a rapidly progressive disease with high mortality. We reviewed randomized controlled trials (RCTs) to evaluate the effect of pulmonary vasodilators on mortality in PAH. Hypothesis: Our objective was to determine the effect of PAH medicines on survival, and the impact of trial design on outcomes. Methods: We conducted a meta-analysis using MEDLINE, EMBASE, Cochrane Library, Web of Science, and Scopus (inceptions-May 2014) of all RCTs treating PAH with pulmonary vasodilators. Data on all-cause mortality and hemodynamics were abstracted. Summary relative risks (RR) and 95% confidence intervals (CI) for outcomes were calculated using a random effects model comparing various pulmonary vasodilators with control. Results: The analysis included 31 RCTs (6,271 patients, 77% female, range of mean age 30-57 years). Median follow up was 12 weeks (18 trials had ≤ 12 weeks follow up, and only 5 followed patients ≥ 6 months). When analyzed together (n = 14), FDA-approved pulmonary vasodilators demonstrated a mortality benefit with RR = 0.77 (95% CI = 0.60 - 099, I 2 = 0%). When analyzed by vasodilator type, prostacyclins were the only class of agents with significant mortality benefit, RR = 0.61 (95% CI 0.38-0.98, I 2 = 1%). Oral pulmonary vasodilators demonstrated no significant benefit on PAH mortality, RR = 0.85 (95% CI 0.65-1.12, I 2 = 0.0%), but we could not exclude a clinically meaningful benefit or harm. Many studies reported ≤1 death in at least one treatment arm. When we limited the analysis to only studies with at least 2 deaths in both treatment arms (n=9), the mortality benefit for pulmonary vasodilators was no longer significant (RR 0.86, CI 0.66-1.12, I 2 =0%), but the point estimate was below 1 and we could not exclude a reduction or increase in risk. For studies with the shorter follow up of ≤ 12 weeks, the RR of mortality for active treatment arm was 0.54 (95% CI 0.35-0.83), compared to trials with > 12 weeks of follow up where the RR of mortality was not significant at 0.91 (95% CI 0.67-1.22). Conclusion: There is an observed mortality benefit for prostacyclins in PAH. For the oral pulmonary vasodilators, there is no observed mortality benefit based on either design of trials, length of follow up or efficacy of the agents studied.

Management of pulmonary hypertension: targeted therapies

ESC CardioMed ◽  
2018 ◽  
pp. 2514-2518
Author(s):  
Nazzareno Galiè ◽  
Alessandra Manes ◽  
Massimiliano Palazzini
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Randomized Controlled Trials ◽  
Exercise Capacity ◽  
Controlled Trials ◽  
Randomized Controlled ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial

Three main signalling pathways are involved in the pathobiology of pulmonary hypertension and are targeted with specific drugs: the endothelin, the nitric oxide–cGMP, and the prostacyclin pathways. The endothelin pathway is over-activated leading to pulmonary vasoconstriction and vessel cells proliferation. Endothelin receptor antagonists are orally available drugs that counteract the endothelin detrimental effects and improve symptoms, exercise capacity, and the outcome of patients with pulmonary arterial hypertension. Phosphodiesterase type 5 inhibitors and guanylate cyclase stimulators are orally available drugs, which promote the activity of the nitric oxide pathway that is downregulated in the pulmonary vessels of pulmonary arterial hypertension patients. Different randomized controlled trials demonstrate favourable effects of these compounds on clinical, exercise, and haemodynamic parameters. Dysregulation of the prostacyclin metabolic pathways has been shown in patients with pulmonary arterial hypertension as assessed by reduction of prostacyclin synthase expression in the pulmonary arteries and of prostacyclin urinary metabolites. The clinical use of prostacyclin in patients with pulmonary arterial hypertension has been extended by the synthesis of stable analogues and by prostacyclin receptor agonists that possess different pharmacokinetic properties but share qualitatively similar pharmacodynamics effects. Prostacyclin analogues are available for intravenous, subcutaneous, inhaled, and oral administration; the prostacyclin receptor agonists are orally available. Multiple randomized controlled trials show variable efficacy of these compounds on symptoms, exercise capacity, haemodynamics, and the outcome of patients with pulmonary arterial hypertension.

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