Abstract:
:In pulmonary hypertension, as in many other diseases, there is a need for a smarter approach to evaluating new treatments. The traditional randomized controlled trial has served medical science well, but constrains the development of treatments for rare diseases. A workshop was established to consider alternative clinical trial designs in pulmonary hypertension and here discusses their merits, limitations and challenges to implementation of novel approaches.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Grieve AP,Chow SC,Curram J,Dawe S,Harnisch LO,Henig NR,Hung HM,Ivy DD,Kawut SM,Rahbar MH,Xiao S,Wilkins MRdoi
10.4103/2045-8932.109933subject
Has Abstractpub_date
2013-01-01 00:00:00pages
217-25issue
1eissn
2045-8932issn
2045-8940pii
PC-3-217journal_volume
3pub_type
杂志文章abstract::Adherence to therapy for pulmonary arterial hypertension is essential to optimize patient outcomes, but data on real-world adherence to different pulmonary arterial hypertension drug classes are limited. This retrospective database analysis evaluated relationships between adherence, hospitalization, and healthcare cos...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019880086
更新日期:2020-03-18 00:00:00
abstract::Inhaled treprostinil (Tyvaso) has been shown to be a safe and effective addition to pulmonary arterial hypertension (PAH) oral therapies; however, the respiratory-related safety profile of inhaled treprostinil required further elucidation in the setting of routine clinical care. The objectives of this study were to ch...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/688059
更新日期:2016-09-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) patients have distinct disease courses and responses to treatment, but current diagnostic and treatment schemes provide limited insight. We aimed to see if cluster analysis could distinguish clinical phenotypes in PAH. An unbiased cluster analysis was performed on 17 baseline clin...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217705891
更新日期:2017-04-01 00:00:00
abstract::Right ventricular (RV) dysfunction in acute respiratory distress syndrome (ARDS) contributes to increased mortality. Our aim is to identify reproducible transthoracic echocardiography (TTE) parameters of RV dysfunction that can be used to predict outcomes in ARDS. We performed a retrospective single-center cohort pilo...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/685549
更新日期:2016-06-01 00:00:00
abstract::Pathological alterations of inspiratory capacity (IC) have been observed in pulmonary hypertension. However, the clinical significance of IC in operable chronic thromboembolic pulmonary hypertension (CTEPH) without other pulmonary diseases remains unknown. CTEPH patients scheduled for pulmonary endarterectomy were pro...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217709763
更新日期:2017-04-01 00:00:00
abstract::Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures. Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression from pulmonary arterial dilatation is rare in adults, and there are ...
journal_title:Pulmonary circulation
pub_type:
doi:10.1086/683687
更新日期:2015-12-01 00:00:00
abstract::Right-ventricular function is a good indicator of pulmonary arterial hypertension (PAH) prognosis; however, how the right ventricle (RV) adapts to the pressure overload is not well understood. Here, we aimed at characterizing the time course of RV early remodeling and discriminate the contribution of ventricular geome...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018800439
更新日期:2018-10-01 00:00:00
abstract::Clinical guidelines for pulmonary hypertension recommend evaluating treatment response through various methods; however, electrocardiography (ECG) is not included as one of the methods of choice. We aimed to identify ECG parameters that correlated with prognosis in patients with pulmonary arterial hypertension (PAH). ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018776496
更新日期:2018-04-01 00:00:00
abstract::Oral treprostinil (TRE) is a prostacylin that is approved for the treatment of patients with pulmonary arterial hypertension (PAH). Dosing is approved for two or three times daily (t.i.d.); however, adverse effects, including gastrointestinal-related symptoms, may limit the ability to reach optimal doses. We report ou...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045893217744512
更新日期:2018-01-01 00:00:00
abstract::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 wa...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217743966
更新日期:2018-01-01 00:00:00
abstract::The present study aimed to propose the pulmonary hypertension for predicting left ventricular dysfunction in adults after patent ductus arteriosus closure. A total of 183 patients (age ≥18 years) after patent ductus arteriosus occlusion were retrospectively collected in this study. In brief, pre-, post-procedure and s...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019888428
更新日期:2019-11-15 00:00:00
abstract::While it is well established that the haemodynamic cause of hypoxic pulmonary hypertension is increased pulmonary vascular resistance, the molecular pathogenesis of the increased resistance remains incompletely understood. Macrophage migration inhibitory factor is a pleiotropic cytokine with endogenous tautomerase enz...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020941352
更新日期:2020-10-26 00:00:00
abstract::Quantifying metabolic derangements in pulmonary hypertension (PH) by plasma metabolomics could identify biomarkers useful for diagnosis and treatment. The objective of this paper is to test the hypotheses that circulating metabolites are differentially expressed in PH patients compared with controls and among differen...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/690554
更新日期:2017-02-01 00:00:00
abstract::Pregnancy is not advised for patients with Pulmonary hypertension (PH) because of high risk of PH crisis. However, some patients have undiagnosed idiopathic pulmonary arterial hypertension (IPAH) before pregnancy. Upfront combination therapy has high efficacy for patients with IPAH. However, some patients are unable t...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045894020910140
更新日期:2020-02-28 00:00:00
abstract::Right ventricular (RV) function is a strong independent predictor of outcome in a number of distinct cardiopulmonary diseases. The RV has a remarkable ability to sustain damage and recover function which may be related to unique anatomic, physiologic, and genetic factors that differentiate it from the left ventricle. ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.101407
更新日期:2012-07-01 00:00:00
abstract::Limited data about the long-term prognosis and response to therapy in pulmonary arterial hypertension patients with World Health Organization functional class I/II symptoms are available. PubMed and Embase were searched for publications of observational registries and randomized, controlled trials in pulmonary arteria...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020935291
更新日期:2020-11-25 00:00:00
abstract::Chronic lung disease (CLD), including pulmonary fibrosis (PF) and chronic obstructive pulmonary disease (COPD), is the fourth leading cause of mortality worldwide. Both are debilitating pathologies that impede overall tissue function. A common co-morbidity in CLD is vasculopathy, characterized by deregulated angiogene...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217739807
更新日期:2018-01-01 00:00:00
abstract::Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of un...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045893217748054
更新日期:2018-01-01 00:00:00
abstract::Pulmonary embolism is associated with high rates of mortality and morbidity. It is important to understand direct comparisons of current interventions to differentiate favorable outcomes and complications. The objective of this study was to compare ultrasound-accelerated thrombolysis versus systemic thrombolysis versu...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020953724
更新日期:2020-09-28 00:00:00
abstract::Riociguat is approved for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Some patients have difficulty swallowing tablets; therefore, 2 randomized, nonblinded, crossover studies compared the relative bioavailability of riociguat oral suspensions and immediate-releas...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/685020
更新日期:2016-03-01 00:00:00
abstract::Patients with pulmonary hypertension are more sedentary than the general population, but attitudes and experiences that may influence their exercise behaviour remain poorly understood. This study identified patterns of behaviour, attitudes towards exercise, barriers and enablers of exercise for people living with pulm...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020968023
更新日期:2020-11-16 00:00:00
abstract::A loss of K+ efflux in pulmonary arterial smooth muscle cells (PASMCs) contributes to abnormal vasoconstriction and PASMC proliferation during pulmonary hypertension (PH). Activation of high-conductance Ca2+-activated (BK) channels represents a therapeutic strategy to restore K+ efflux to the affected PASMCs. However,...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/688838
更新日期:2016-12-01 00:00:00
abstract::Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not well understood an...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045893217699228
更新日期:2017-04-01 00:00:00
abstract::Acute respiratory distress syndrome in patients with Coronavirus disease 19 is associated with an unusually high incidence of pulmonary embolism and microthrombotic disease, with evidence for reduced fibrinolysis. We describe seven patients requiring invasive ventilation for COVID-19-associated acute respiratory distr...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020973906
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abstract::Endothelial dysfunction plays an important role in the pathogenesis of pulmonary arterial hypertension (PAH) in sickle cell disease (SCD). A variety of evidence suggests that circulating endothelial progenitor cells (EPCs) play an integral role in vascular repair. We hypothesized that SCD patients with PAH are deficie...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.94834
更新日期:2012-01-01 00:00:00
abstract::Transforming growth factor-β (TGF-β) inhibition is an investigational therapy for pulmonary arterial hypertension with promising results in experimental studies. The present work compared this approach with endothelin-receptor blockade and evaluated the effects of combined administration. Pulmonary arterial hypertensi...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.105034
更新日期:2012-10-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is a major complication of sickle cell disease (SCD). Low levels of apolipoprotein A1 (Apo-A1) have been implicated in the development of PAH in SCD. We speculate that lower levels of Apo-A1 are related to dysregulation of the ubiquitin-proteasome pathway (UPP). Of 36 recruited pa...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674763
更新日期:2013-12-01 00:00:00
abstract::There are few data on the epidemiology of pulmonary hypertension (PH)-related hospitalizations in children in the United States. Our aim was to determine hospital mortality, length of hospitalization, and hospital charges pertaining to PH-related hospitalizations and also the effects of codiagnoses and comorbidities. ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/681226
更新日期:2015-06-01 00:00:00
abstract::Pulmonary hypertension currently has minimal guidelines for outpatient disease management. Congestive heart failure studies, however, have shown effectiveness of disease management plans in reducing all-cause mortality and all-cause and congestive heart failure-related hospital readmissions. Heart failure exacerbation...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020968471
更新日期:2020-12-07 00:00:00
abstract::Vasculopathies, characterized by the formation of fragile and abnormal microvessels, are associated with the severity of many chronic lung diseases, including pulmonary fibrosis, emphysema/chronic obstructive pulmonary disease, systemic sclerosis, and hypertension. However, the study of human lung vasculature has been...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217702346
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