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 often act as a central component of the team. This survey aimed to evaluate the current organization of PH clinics and the role of nurses. The survey (35 questions) was online February-December 2015 and was advertised at international PH nurse meetings and through international PH organizations to their corresponding clinics. In total, 126 healthcare professionals from 32 countries responded. According to respondents, 54% of clinics managed >200 patients, of whom 49% had a pulmonary arterial hypertension (PAH) diagnosis, on average. In terms of staff, 66% had a dedicated program administrator, 35% had one full-time nurse coordinator/practitioner/specialist, and 57% had a nurse attend outpatient clinic alongside a physician. Crucially, not all centers had a nurse in their team. The role of a nurse coordinator/practitioner/specialist varied with 51% taking patient histories/examinations and 66% managing outpatients. In 34% of clinics, nurses were involved in their own research. Protocols were available for PH therapies (81%), management of heart failure (37%) and pain (26%), and referring patients who did not have PAH/chronic thromboembolic PH back to their specialist (62%). Not all clinics are meeting all of the standards outlined in the latest guidelines with key areas of improvement being level of support from/for nurses, clear protocols, and referral pathways.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Doyle-Cox C,Nicholson G,Stewart T,Gin-Sing Wdoi
10.1177/2045894019855611subject
Has Abstractpub_date
2019-04-01 00:00:00pages
2045894019855611issue
2eissn
2045-8932issn
2045-8940journal_volume
9pub_type
杂志文章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::Pulmonary arterial hypertension (PAH) is a rapidly progressive and fatal disease for which there is an ever-expanding body of genetic and related pathophysiological information on disease pathogenesis. The most common single culprit gene known is BMPR2, and animal models of the disease in several forms exist. There is...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.87293
更新日期:2011-07-01 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::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 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::Schistosomiasis-associated pulmonary arterial hypertension (PAH) is one of the most common causes of pulmonary hypertension worldwide. A potential contributing mechanism to the pathogenesis of this disease is a localized immune reaction to retained and persistent parasite-derived antigens. We sought to identify Schist...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.4103/2045-8932.93544
更新日期:2011-10-01 00:00:00
abstract::Pulmonary hypertension (PH) comprises a group of pathophysiological syndromes characterized by elevated pulmonary artery pressure and pulmonary vascular resistance, which lead to right ventricular overload, and even right heart failure. PH has a poor prognosis and severely leads to a decline in quality of life. Histor...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1177/2045894020937129
更新日期:2020-07-07 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::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::Pulmonary hypertension is recognized as a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). We now report benchtop phenotyping from the explanted lungs of the first successful lung transplant in SCD. Pulmonary artery smooth muscle cells (PASMCs) cultured from the explanted lungs were...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/674754
更新日期:2013-12-01 00:00:00
abstract::Pulmonary hypertension is a multifactorial disease with a high morbidity and mortality. Right ventricular function is the most important predictor of morbidity and mortality in patients suffering from pulmonary hypertension, but currently there are no approved treatments directly supporting the failing right ventricle...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018790905
更新日期:2018-07-01 00:00:00
abstract::Hypereosinophilic syndrome is a myeloproliferative disorder characterized by persistent eosinophilia with involvement of multiple organs. The occurrence of severe pulmonary hypertension (PH) in the setting of hypereosinophilic syndrome is very uncommon. A 43-year-old man with documented idiopathic hypereosinophlic syn...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045894018793999
更新日期:2019-01-01 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::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::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::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::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::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::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::Pharmacokinetic studies with oral treprostinil demonstrate that three times daily (TID) dosing reduces peak-to-trough plasma trepostinil fluctuations compared with twice daily (BID) dosing. TID dosing may allow for faster titration, higher total daily doses, and potentially improve the tolerability of oral trepostinil...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018770654
更新日期:2018-04-01 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::Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is associated with worse outcome than idiopathic pulmonary arterial hypertension (IPAH), potentially due to worse right ventricular adaptation to load as suggested by pressure-volume loop analysis. The value of non-invasive load-adaptability metrics has ...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894018788268
更新日期:2018-07-01 00:00:00
abstract::Cardiac magnetic resonance-derived ventricular variables are predictive of mortality in pulmonary arterial hypertension. Rodent models which emphasize ventricular function, allowing serial monitoring, are needed to identify pathophysiological features and novel therapies for pulmonary arterial hypertension. We investi...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019897513
更新日期:2020-02-10 00:00:00
abstract::Carboxyl terminus of Hsc70-interacting protein (CHIP) is a 35-kDa cytoplasmic protein expressed in human striated muscle, brain, aortic smooth muscle, endothelial cells, and other tissues. Studies have confirmed that CHIP regulates cell growth, apoptosis, cell phenotype, metabolism, neurodegeneration, etc. However, wh...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1177/2045894019875343
更新日期:2019-09-09 00:00:00
abstract::Pulmonary arterial hypertension can be associated with exposure to certain drugs or toxins. However, only a few cases of drug-induced pulmonary arterial hypertension have been previously reported. Anagrelide is an oral imidazoquinazoline agent that is prescribed for reducing elevated platelet counts in patients with m...
journal_title:Pulmonary circulation
pub_type:
doi:10.1177/2045894019896682
更新日期:2019-12-27 00:00:00
abstract::[This corrects the article DOI: 10.1086/688060.]. ...
journal_title:Pulmonary circulation
pub_type: 已发布勘误
doi:10.1086/689215
更新日期:2016-12-01 00:00:00
abstract::Andeans and Tibetans have less altitude reduction in birth weight than do shorter-resident groups, but only Tibetans are protected from pulmonary hypertension and chronic mountain sickness (CMS). We hypothesized that differences in neonatal oxygenation were involved, with arterial O2 saturation (SaO2) being highest in...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.1086/679719
更新日期:2015-03-01 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:: ...
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::Most subtypes of pulmonary arterial hypertension (PAH) are characterized by a greater susceptibility to disease among females, although females with PAH appear to live longer after diagnosis. While this "estrogen paradoxȍ of enhanced female survival despite increased female susceptibility remains a mystery, recent pro...
journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
doi:10.4103/2045-8932.114756
更新日期:2013-04-01 00:00:00