Sendaway capillary NT-proBNP in pulmonary hypertension.

Background: N-terminal pro-B-type natriuretic peptide (NT-proBNP) is a biomarker of cardiac ventricular wall stress that is incorporated into pulmonary hypertension (PH) risk stratification models. Sendaway sampling may enable patients to perform NT-proBNP tests remotely. This UK-wide study aimed to assess the agreement of sendaway NT-proBNP with standard venous NT-proBNP and to assess the effect of delayed processing.

European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology.

Aims: To develop a suite of quality indicators (QIs) for the evaluation of the care and outcomes for adults with pulmonary arterial hypertension (PAH).

Methods And Results: We followed the European Society of Cardiology (ESC) methodology for the development of QIs. This included (i) the identification of key domains of care for the management of PAH, (ii) the proposal of candidate QIs following systematic review of the literature, and (iii) the selection of a set of QIs using a modified Delphi method.

Establishing expert consensus for the optimal approach to holistic risk-management in pulmonary arterial hypertension: a Delphi process and narrative review.

: Pulmonary arterial hypertension (PAH) is associated with significant morbidity and reduced life expectancy. Various medical therapies, together with non-medical therapies such as exercise training, have been shown to improve outcomes for patients. We performed a Delphi consensus process to establish optimal approaches to optimizing patient care.

EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study.

Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified.

The practical management of fluid retention in adults with right heart failure due to pulmonary arterial hypertension.

Our aim with this review is to provide practical advice and management support for nurses and other healthcare practitioners in managing fluid retention in adults with right heart failure (RHF) due to pulmonary arterial hypertension (PAH). Vigilant management of RHF is important for maintaining patient quality of life, as fluid overload can lead to abdominal bloating (ascites) and peripheral oedema, which also has a major impact on patients' morbidity and mortality. Patients with RHF should be assessed regularly for signs of fluid retention.

Current organization of specialist pulmonary hypertension clinics: results of an international survey.

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 patient experience of pulmonary hypertension: a large cross-sectional study of UK patients.

Background: Pulmonary Hypertension Association UK (PHA-UK) is the only charity in the UK especially for people affected by pulmonary hypertension (PH). To better understand the impact of PH on patients and carers beyond clinical symptoms, the PHA-UK carried out a cross-sectional survey on the effect of PH on daily living, along with a follow-up survey assessing the financial impact of PH.

Methods: This is a descriptive cross-sectional survey of adult patients with PH in the UK.

Elicitation of health state utilities associated with the mode of administration of drugs acting on the prostacyclin pathway in pulmonary arterial hypertension.

Introduction: Pulmonary arterial hypertension (PAH) is a rare, incurable disease associated with decreased life expectancy and a marked impact on quality of life (QoL). There are three classes of drugs available for treatment: endothelin receptor antagonists (ERA), drugs acting on nitric oxide pathway (riociguat and phosphodiesterase type 5 inhibitors [PDE5i]), and drugs acting on prostacyclin pathway. The latter have widely different modes of administration - continuous intravenous infusion, continuous subcutaneous infusion, inhaled, and oral - each associated with variable treatment burden, and implications for health economic assessment.

Palliative care in pulmonary arterial hypertension.

Purpose Of Review: Developments in the management of pulmonary arterial hypertension have significantly improved prognosis changing this from an acute to a chronic disease. Despite optimal treatment many patients still have a high-symptom burden both because of the disease and the side-effects of therapy, consequently there is an increasing need for a palliative care approach to improve the quality of life for this patient group. This review article will outline the need for palliative care support for patients with pulmonary arterial hypertension, discuss the barriers that currently exist and suggest how this may be improved.

Practical considerations for therapies targeting the prostacyclin pathway.

Therapies that target the prostacyclin pathway play a key role in the treatment of both early- and late-stage pulmonary arterial hypertension, and provide significant clinical benefits for patients. A number of agents have been approved, which are administered via intravenous, subcutaneous, inhaled or oral routes. The use of these therapies is associated with practical challenges, relating to the need for up-titration and their routes of administration.

Exercise physiological responses to drug treatments in chronic thromboembolic pulmonary hypertension.

We tested the hypothesis that patients with chronic thromboembolic pulmonary hypertension (CTEPH) that was deemed to be inoperable were more likely to respond to drugs for treating pulmonary arterial hypertension (PAH) by using cardiopulmonary exercise (CPX) testing than those with CTEPH that was deemed to be operable. We analyzed CPX testing data of all patients with CTEPH who were treated with PAH drugs and had undergone CPX testing before and after treatment at a single pulmonary hypertension center between February 2009 and March 2013. Suitability for pulmonary endarterectomy (PEA) was decided by experts in PEA who were associated with a treatment center.

Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: two case reports.

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 no reports in the literature of its occurrence in idiopathic pulmonary arterial hypertension.

Response to pulmonary arterial hypertension drug therapies in patients with pulmonary arterial hypertension and cardiovascular risk factors.

The age at diagnosis of pulmonary arterial hypertension (PAH) and the prevalence of cardiovascular (CV) risk factors are increasing. We sought to determine whether the response to drug therapy was influenced by CV risk factors in PAH patients. We studied consecutive incident PAH patients (n = 146) between January 1, 2008, and July 15, 2011.

Diagnosing and managing pulmonary hypertension.

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure > or = 25 mmHg at rest as assessed invasively by right heart catheterisation. It can affect patients at any age and presents with non-specific symptoms. Accurate diagnosis is important as while PH is a potentially lethal disease it is treatable.

The association of clinical outcome with right atrial and ventricular remodelling in patients with pulmonary arterial hypertension: study with real-time three-dimensional echocardiography.

Aims: Right atrial (RA) dilatation may be important for patients' outcome in pulmonary arterial hypertension (PAH). The aim of this study was to examine the longitudinal RA and right ventricular (RV) remodelling in PAH patients using real-time three-dimensional echocardiography (3DE) and their relation to clinical outcome.

Methods And Results: Sixty-two consecutive PAH patients were studied and compared with a control group of 30 healthy volunteers.

Pulmonary arterial hypertension: a multidisciplinary approach to care.

Pulmonary arterial hypertension (PAH) is a devastating disease that has a considerable effect on quality of life and eventually leads to right heart failure. The past ten years have seen improvements in drug therapy and patient care, and the prospects for patients with PAH are now considerably better. Although new drugs have played a major role in this improvement, the focus is on improving the patient care aspect of PAH management.

A clinical comparison of slow- and rapid-escalation treprostinil dosing regimens in patients with pulmonary hypertension.

Background And Objective: Subcutaneous treprostinil is an effective treatment for pulmonary arterial hypertension (PAH). A previous pivotal study indicated that infusion site pain was dose dependent and resulted in suboptimal dose escalation by week 12 and a reduced clinical benefit. We hypothesized that a rapid-escalation treprostinil dosing regimen would be as safe and effective as a slow-escalation dosing regimen.

Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension.

Unlabelled: Pulmonary hypertension (PH) is a progressive disease with a poor prognosis. Identifying chronic thromboembolic pulmonary disease as a cause of PH has major clinical implications as these patients could be potentially offered a surgical cure. Ventilation-perfusion (V/Q) scintigraphy has a high sensitivity to detect embolic disease but its value has been challenged with the emergence of multidetector CT pulmonary angiography (CTPA).

Sildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) study.

Rationale: Phosphodiesterase type 5 (PDE5) inhibition has been proposed for the treatment for pulmonary arterial hypertension (PAH).

Objective: This study compared adding sildenafil, a PDE5 inhibitor, to conventional treatment with the current practice of adding bosentan, an endothelin receptor antagonist.

Methods: Twenty-six patients with PAH, idiopathic or associated with connective tissue disease, World Health Organization (WHO) functional class III, were randomized in a double-blind fashion to receive sildenafil (50 mg twice daily for 4 weeks, then 50 mg three times daily) or bosentan (62.