Abstract:
:Adults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pulmonary hypertension. The only approved medical therapy for chronic thromboembolic pulmonary hypertension is riociguat. We report the experience, safety and tolerability of riociguat use in a series of sickle cell disease patients with chronic thromboembolic pulmonary hypertension.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Weir NA,Conrey A,Lewis D,Mehari Adoi
10.1177/2045894018791802subject
Has Abstractpub_date
2018-10-01 00:00:00pages
2045894018791802issue
4eissn
2045-8932issn
2045-8940journal_volume
8pub_type
abstract::Despite recent advances, the prognosis of pulmonary hypertension (PH) remains poor. While the initial insult in PH implicates the pulmonary vasculature, the functional state, exercise capacity, and survival of such patients are closely linked to right ventricular (RV) function. In the current study, we sought to inves...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019862435
更新日期:2019-07-01 00:00:00
abstract::PH occurs alone or in association with many disorders. Many patients with transthoracic echocardiography (TTE) findings suggesting PH never receive additional evaluation. Patient characteristics and echocardiographic data associated with increased recognition of PH have not been fully evaluated. We evaluated TTE repor...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.113184
更新日期:2013-04-01 00:00:00
abstract::Microparticle release by vascular endothelium has been implicated in various cardiovascular pathologies. Ventilator-induced lung injury (VILI) is a life-threatening complication of mechanical ventilation at high tidal volumes associated with excessive mechanical stretch of pulmonary vascular endothelial cells. However...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.109921
更新日期:2013-01-01 00:00:00
abstract::To shed light on the efficacy of oxygen therapy in pulmonary arterial hypertension (PAH) when hypoxemia is not present, we report seven years of observational data for a female patient recorded between February 2012 and February 2019 when she was aged 7.8-14.8 years. The patient was born with an atrial septal defect (...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019845610
更新日期:2019-04-01 00:00:00
abstract::Right ventricular failure (RVF) is the most important prognostic factor for both morbidity and mortality in pulmonary arterial hypertension (PAH), but also occurs in numerous other common diseases and conditions, including left ventricle dysfunction. RVF remains understudied compared with left ventricular failure (LVF...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217714463
更新日期:2017-07-01 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::Increased exposure to ambient particulate matter (PM) is associated with elevated morbidity and mortality in patients with cardiopulmonary diseases and cancer. We and others have shown that PM induces lung microvascular barrier dysfunction which potentially enhances the systemic toxicity of PM. However, the mechanisms...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/689906
更新日期:2017-07-01 00:00:00
abstract::Patients with chronic thromboembolic pulmonary hypertension (CTEPH) require lifelong anticoagulation therapy. However, the bleeding risk and recurrence of venous thromboembolism (VTE) in CTEPH patients who are administered warfarin have not been adequately evaluated. The purpose of this study was to evaluate the risk ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217717258
更新日期:2017-07-01 00:00:00
abstract::Despite the discovery more than 15 years ago that patients with hereditary pulmonary arterial hypertension (HPAH) inherit BMP type 2 receptor ( BMPR2) mutations, it is still unclear how these mutations cause disease. In part, this is attributable to the rarity of HPAH and difficulty obtaining tissue samples from patie...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018765840
更新日期:2018-04-01 00:00:00
abstract::There are limited data on the management of pulmonary arterial hypertension (PAH) in the elderly; therefore, this analysis compared the safety and efficacy of tadalafil between patients ≥65 and <65 years old. This was a post hoc analysis of the randomized, double-blind, placebo-controlled phase 3 Pulmonary Arterial Hy...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674901
更新日期:2014-03-01 00:00:00
abstract::Our aim is to assess the safety and potential efficacy of a novel treatment paradigm in pulmonary arterial hypertension (PAH), immunomodulation by blocking interleukin-6 (IL6) signaling with the IL6 receptor antagonist, tocilizumab. Inflammation and autoimmunity are established as important in PAH pathophysiology. One...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217735820
更新日期:2018-01-01 00:00:00
abstract::The aim of this study was to investigate whether a dual endothelin receptor antagonist bosentan modulates the kinetics of bone marrow-derived stem cells in inhibiting the development of pulmonary hypertension. Bone marrow chimeric mice, transplanted with enhanced green fluorescent protein (eGFP)-positive bone marrow m...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020919355
更新日期:2020-05-14 00:00:00
abstract:: ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019865158
更新日期:2019-07-03 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::Uncorrected atrial septal defect undergoes right ventricle chronic volume overload which may lead to pulmonary hypertension and Eisenmenger Syndrome. The soluble suppression of tumorigenicity-2 is a left ventricle strain biomarker; however, its role in right ventricle strain is unclear. This study aimed to investigate...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020915832
更新日期:2020-05-26 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::Many dyspneic patients who undergo computerized tomographic pulmonary angiography (CTPA) for presumed acute pulmonary embolism (PE) have no identified cause for their dyspnea yet have persistent symptoms, leading to more CTPA scanning. Right ventricular (RV) dysfunction or overload can signal treatable causes of dyspn...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/679723
更新日期:2015-03-01 00:00:00
abstract::South America is a territory of 17,819,100 km(2), where ∼388 million people live in 13 countries. In the region, access to medical assistance (e.g., for treatment of cardiovascular disorders) is relatively easy in metropolitan areas but difficult in remote places such as the Andes and the Amazon. Altitudes up to ∼6,70...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/676747
更新日期:2014-09-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is characterized by obstruction of pre-capillary pulmonary arteries, which leads to sustained elevation of pulmonary arterial pressure. Identifying those at risk through early interventions, such as genetic testing, may mitigate disease course. Current practice guidelines recommen...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045893217700156
更新日期:2017-04-01 00:00:00
abstract::Endothelial nitric oxide synthase (eNOS) plays a major role in maintaining pulmonary vascular homeostasis. Tetrahydrobiopterin (BH4), an essential cofactor that stabilizes the dimerization of eNOS and balances nitric oxide (NO) and superoxide production, may have therapeutic potential in pulmonary hypertension. In the...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/677361
更新日期:2014-09-01 00:00:00
abstract::Increased peripheral resistance of small distal pulmonary arteries is a hallmark signature of pulmonary hypertension (PH) and is believed to be the consequence of enhanced vasoconstriction to agonists, thickening of the arterial wall due to remodeling, and increased thrombosis. The elevation in arterial tone in PH is ...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/680189
更新日期:2015-06-01 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
更新日期:2017-03-27 00:00:00
abstract::In 301 treatment-naïve patients with pulmonary arterial hypertension stratified by the European Society of Cardiology/European Respiratory Society risk score, further stratification of intermediate-risk patients based on six-minute walk distance and the tricuspid annular plane systolic excursion/systolic pulmonary art...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894020961739
更新日期:2020-10-07 00:00:00
abstract::Obesity is causally linked to a number of comorbidities, including cardiovascular disease, diabetes, renal dysfunction, and cancer. Obesity has also been linked to pulmonary disorders, including pulmonary arterial hypertension (PAH). It was long believed that obesity-related PAH was the result of hypoventilation and h...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/678510
更新日期:2014-12-01 00:00:00
abstract::Echocardiography, a non-invasive and cost-effective method for monitoring cardiac function, is commonly used for evaluation and pre-clinical diagnostics of pulmonary hypertension (PH). Previous echocardiographic studies in experimental models of PH are fragmentary in terms of the evaluation of right ventricle (RV) fun...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019841987
更新日期:2019-04-01 00:00:00
abstract::The maintenance of endothelial barrier integrity is absolutely essential to prevent the vascular leak associated with pneumonia, pulmonary edema resulting from inhalation of toxins, acute elevation to high altitude, traumatic and septic lung injury, acute lung injury (ALI), and its life-threatening complication, acute...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018773044
更新日期:2018-04-01 00:00:00
abstract::Optimal pulmonary hypertension (PH) management relies on a timely, accurate diagnosis and follow-up in specialized clinics by multidisciplinary teams that have clearly defined responsibilities and protocols. Internationally agreed criteria for expert center staff are lacking, particularly with respect to nurses, who o...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019855611
更新日期:2019-04-01 00:00:00
abstract::Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition characterized by single or recurrent pulmonary thromboemboli, which promote pulmonary vascular remodeling. MicroRNA (miRNA), is a small, noncoding RNA that is involved in multiple cell processes and functions and may partici...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674310
更新日期:2013-09-01 00:00:00
abstract::The development of pulmonary arterial hypertension (PAH) in pediatric patients has been linked to the production of the arachidonic acid metabolite, thromboxane A(2) (TxA(2)). The present study evaluated the therapeutic effect of furegrelate sodium, a thromboxane synthase inhibitor, on the development of PAH in a neon...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.97605
更新日期:2012-04-01 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