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Pulmonary Arterial Hypertension

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1. Prostacyclin for pulmonary arterial hypertension. (PubMed)

Prostacyclin for pulmonary arterial hypertension. Pulmonary arterial hypertension (PAH) is characterised by pulmonary vascular changes, leads to elevated pulmonary artery pressures, dyspnoea, a reduction in exercise tolerance, right heart failure, and ultimately death.Prostacyclin analogue drugs mimic endogenous prostacyclin which leads to vasodilation, inhibition of platelet aggregation, and reversal of vascular remodelling. Prostacyclin's short half-life theoretically enhances selectivity (...) cardiopulmonary haemodynamics (reduction in mean pulmonary artery pressure by 3.60 mmHg (95% CI -4.73 to -2.48); pulmonary vascular resistance by 2.81 WU (95% CI -3.80 to -1.82); right atrial pressure by 1.90 mmHg (95% CI -2.58 to -1.22), and increase in cardiac index by 0.31 L/min/m2 (95% CI 0.23 to 0.38); low-certainty evidence), improved dyspnoea (low-certainty evidence, and improved quality of life (moderate-certainty evidence), when compared to control. When only subcutaneous/inhaled trials were included

2019 Cochrane

2. Natriuretic Peptide Testing for Pulmonary Arterial Hypertension: Clinical Effectiveness, Cost-Effectiveness, and Guidelines

Natriuretic Peptide Testing for Pulmonary Arterial Hypertension: Clinical Effectiveness, Cost-Effectiveness, and Guidelines Natriuretic Peptide Testing for Pulmonary Arterial Hypertension: Clinical Effectiveness, Cost-Effectiveness, and Guidelines | CADTH.ca Find the information you need Natriuretic Peptide Testing for Pulmonary Arterial Hypertension: Clinical Effectiveness, Cost-Effectiveness, and Guidelines Natriuretic Peptide Testing for Pulmonary Arterial Hypertension: Clinical (...) Effectiveness, Cost-Effectiveness, and Guidelines Last updated: July 24, 2019 Project Number: RB1358-000 Product Line: Research Type: Devices and Systems Report Type: Summary of Abstracts Result type: Report Question What is the clinical utility of natriuretic peptide testing for prognosis or guiding therapy for pulmonary arterial hypertension? What is the cost-effectiveness of natriuretic peptide testing for prognosis or guiding therapy for pulmonary arterial hypertension? What are the guidelines

2019 Canadian Agency for Drugs and Technologies in Health - Rapid Review

3. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension Current pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension (PAH) in the French, Swedish and COMPERA registries. Our (...) objective was to investigate the three abbreviated risk stratification methods for patients with mostly prevalent PAH and chronic thromboembolic pulmonary hypertension (CTEPH), in patients from the PATENT-1/2 and CHEST-1/2 studies of riociguat.Risk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.With all three methods, riociguat improved risk group/strata in patients with PAH after 12

2019 EvidenceUpdates

6. Idiopathic pulmonary arterial hypertension

Idiopathic pulmonary arterial hypertension Idiopathic pulmonary arterial hypertension - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Idiopathic pulmonary arterial hypertension Last reviewed: February 2019 Last updated: May 2018 Summary A rare usually fatal disease most commonly seen in women that is best managed in centres with expertise in pulmonary hypertension. Presenting symptoms and signs are non-specific (...) endothelin receptor antagonists, oral phosphodiesterase type-5 inhibitors, the soluble guanylate cyclase stimulator riociguat, and the selective prostacyclin IP receptor agonist selexipag. Prognosis is not good, as most patients eventually develop right ventricular failure. Definition Idiopathic pulmonary arterial hypertension (IPAH) is a disease of the small pulmonary arteries characterised by vascular proliferation and remodelling. It results in a progressive increase in pulmonary vascular resistance

2018 BMJ Best Practice

7. Endothelin receptor antagonists for pulmonary arterial hypertension

Endothelin receptor antagonists for pulmonary arterial hypertension '); } else { document.write(' '); } ACE | Endothelin receptor antagonists for treating pulmonary arterial hypertension Search > > Endothelin receptor antagonists for treating pulmonary arterial hypertension - Endothelin receptor antagonists for treating pulmonary arterial hypertension Published on 2 September 2019 Guidance Recommendations The Ministry of Health's Drug Advisory Committee has recommended: Ambrisentan 5 mg and 10 (...) mg tablets for treating adults with a confirmed diagnosis of WHO Functional Class II or III pulmonary arterial hypertension (PAH) who have one of the following PAH aetiologies: Idiopathic PAH; Heritable or familial PAH; PAH associated with connective tissue disease; Anorexigen-induced PAH; or PAH associated with HIV infection. Subsidy status Ambrisentan 5 mg and 10 mg tablets are recommended for inclusion on the Medication Assistance Fund (MAF) for the abovementioned indication. MAF assistance

2019 Appropriate Care Guides, Agency for Care Effectiveness (Singapore)

8. Therapy for Pulmonary Arterial Hypertension in Adults: Update of the CHEST Guideline and Expert Panel Report

Therapy for Pulmonary Arterial Hypertension in Adults: Update of the CHEST Guideline and Expert Panel Report Therapy for Pulmonary Arterial Hypertension in Adults - CHEST Follow CHEST: Email/Username: Password: Remember me Search Terms Search within Search Share this page Volume 155, Issue 3, Pages 565–586 To read this article in full, please review your options for gaining access at the bottom of the page. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline (...) . In these situations, other factors, such as patient preferences & values, cost, and insurance coverage, may guide decision-making. CCB = calcium channel blocker; 6MWD = 6-min walk distance; FC = functional class; PAH = pulmonary arterial hypertension; PH = pulmonary hypertension; RV = right ventricular; WHO = World Health Organization. Figure 1 Guideline algorithm for pharmacologic therapy for PAH in adults. Where multiple drug options are provided, there is no comparative effectiveness data to suggest greater

2019 American College of Chest Physicians

9. Gasometric gradients between blood obtained from the pulmonary artery wedge and pulmonary artery positions in pulmonary arterial hypertension. (PubMed)

Gasometric gradients between blood obtained from the pulmonary artery wedge and pulmonary artery positions in pulmonary arterial hypertension. Little is known on the pulmonary gradients of oxyhemoglobin, carboxyhemoglobin and methemoglobin in pulmonary arterial hypertension (PAH). We sought to determine these gradients in group 1 PAH and assess their association with disease severity and survival.During right heart catheterization (RHC) we obtained blood from pulmonary artery (PA) and pulmonary (...) artery wedge (PAW) positions and used co-oximetry to test their gasometric differences.We included a total of 130 patients, 65 had group 1 PAH, 40 had pulmonary hypertension (PH) from groups 2-5 and 25 had no PH during RHC. In all groups, PAW blood had higher pH, carboxyhemoglobin and lactate as well as lower pCO2 than PA blood. In group 1 PAH (age 58 ± 15 years, 72% females), methemoglobin in the PAW was lower than in the PA blood (0.83% ± 0.43 vs 0.95% ± 0.50, p = 0.03) and was directly associated

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2019 Respiratory research

10. Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease. (PubMed)

Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease. The purpose of this study was to evaluate the pathological changes of the pulmonary arterioles in patients ≥ 2 years of age who first underwent a pulmonary artery banding (PAB) procedure, followed by bidirectional Glenn or Fontan according to their specific conditions. This was a prospective study of 15 children diagnosed (...) and treated with PAB at the Department of Cardiothoracic Surgery of Anzhen Hospital between January 2009 and December 2012. The percentage of media area (%MS) of pulmonary arteriole, the percentage of media thickness (%MT), and pulmonary arterial density (APSC) were calculated from lung tissue sections. Pulmonary artery pressure decreased significantly after PAB surgery (P < 0.01). Postoperative mean pulmonary artery pressure declined significantly (P < 0.01), the cardiothoracic ratio was reduced (P

2019 Pediatric Cardiology

11. The multiple systemic artery to pulmonary artery fistulas resulting in severe irreversible pulmonary arterial hypertension in patient with previous history of pneumothorax. (PubMed)

The multiple systemic artery to pulmonary artery fistulas resulting in severe irreversible pulmonary arterial hypertension in patient with previous history of pneumothorax. Systemic artery to pulmonary artery fistulas (SA-PAFs), are extremely rare in people without congenital heart disease. In this group of patients pulmonary arterial hypertension was reported in the single case. Then, we describe a case of multiple SA-PAFs, which were the cause of severe nonreversible arterial pulmonary (...) hypertension in a patient who had a right-sided pneumothorax 35 years earlier.52-year-old male Caucasian patient with echocardiographically confirmed pulmonary hypertension (PH) was admitted to cardiology department due to exertional dyspnea and signs of right ventricle failure. Routine screening for causes of secondary PH was negative. Right heart catheterization (RHC) confirmed a high degree arterial PH [mean pulmonary artery pressure (mPAP); 50,6 mmHg, pulmonary wedge pressure (PWP); 11,3 mmHg

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2019 BMC pulmonary medicine

12. Clinical Commissioning Policy: Selexipag for treating pulmonary arterial hypertension

Clinical Commissioning Policy: Selexipag for treating pulmonary arterial hypertension NHS England » Clinical Commissioning Policy: Selexipag for treating pulmonary arterial hypertension (all ages) Search Search Menu Clinical Commissioning Policy: Selexipag for treating pulmonary arterial hypertension (all ages) Document first published: 6 July 2018 Page updated: 6 July 2018 Topic: , Publication type: This policy document outlines the arrangements for funding of treatment for pulmonary arterial (...) hypertension. Document 19 pages

2018 NHS England

13. Selexipag (Uptravi) - for the long-term treatment of pulmonary arterial hypertension (PAH)

Selexipag (Uptravi) - for the long-term treatment of pulmonary arterial hypertension (PAH) Cost-effectiveness of selexipag (Uptravi®) for the long-term treatment of pulmonary arterial hypertension (PAH) in adult patients with WHO functional class (FC) II –III, either as combination therapy in patients insufficiently controlled with an endothelin receptor antagonist (ERA) and/or a phosphodiesterase type 5 (PDE-5) inhibitor, or as monotherapy in patients who are not candidates for these therapies (...) and economic evidence in support of selexipag, long-term treatment of pulmonary arterial hypertension (PAH) in adult patients with WHO functional class (FC) II–III, either as combination therapy in patients insufficiently controlled with an endothelin receptor antagonist (ERA) and/or a phosphodiesterase type 5 (PDE-5) inhibitor, or as monotherapy in patients who are not candidates for these therapies. Selexipag is a selective prostacyclin receptor agonist distinct from prostacyclin and its analogues

2018 Pediatric Endocrine Society

14. Continuous subcutaneous treprostinil for treatment of pulmonary arterial hypertension

Continuous subcutaneous treprostinil for treatment of pulmonary arterial hypertension Continuous subcutaneous treprostinil for treatment of pulmonary arterial hypertension Continuous subcutaneous treprostinil for treatment of pulmonary arterial hypertension HAYES, Inc Record Status This is a bibliographic record of a published health technology assessment. No evaluation of the quality of this assessment has been made for the HTA database. Citation HAYES, Inc. Continuous subcutaneous (...) treprostinil for treatment of pulmonary arterial hypertension. Lansdale: HAYES, Inc. Healthcare Technology Brief Publication. 2017 Authors' conclusions Pulmonary arterial hypertension (PAH) refers to the vascular remodeling of the small pulmonary arteries that is associated with elevated pulmonary arterial pressure (PAP) and eventually right ventricular failure (RVF). Symptoms of PAH begin as shortness of breath at exertion, later progressing to dyspnea with normal activities, and ultimately dyspnea

2017 Health Technology Assessment (HTA) Database.

15. Macitentan (pulmonary arterial hypertension) - Benefit assessment according to õ35a Social Code Book V

Macitentan (pulmonary arterial hypertension) - Benefit assessment according to õ35a Social Code Book V Macitentan (pulmonal arterielle Hypertonie): Nutzenbewertung gemäß § 35a SGB V; Dossierbewertung; Auftrag A16-67 [Macitentan (pulmonary arterial hypertension) - Benefit assessment according to §35a Social Code Book V] Macitentan (pulmonal arterielle Hypertonie): Nutzenbewertung gemäß § 35a SGB V; Dossierbewertung; Auftrag A16-67 [Macitentan (pulmonary arterial hypertension) - Benefit (...) . [Macitentan (pulmonary arterial hypertension) - Benefit assessment according to §35a Social Code Book V] Cologne: Institut fuer Qualitaet und Wirtschaftlichkeit im Gesundheitswesen (IQWiG). IQWiG-Berichte 476. 2017 Final publication URL Indexing Status Subject indexing assigned by CRD MeSH Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Pyrimidines; Sulfonamides Language Published German Country of organisation Germany English summary There is no English language summary

2017 Health Technology Assessment (HTA) Database.

16. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model

Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model The 2015 European pulmonary hypertension (PH) guidelines propose a risk stratification strategy for patients with pulmonary arterial hypertension (PAH). Low-, intermediate- and high-risk strata are defined by estimated 1-year mortality risks of <5%, 5-10% and >10%, respectively. This risk assessment strategy awaits validation.We analysed data from patients (...) with newly diagnosed PAH enrolled into COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension), a European-based PH registry. An abbreviated version of the risk assessment strategy proposed by the European PH guidelines was applied, using the following variables: World Health Organization functional class, 6-min walking distance, brain natriuretic peptide or its N-terminal fragment, right atrial pressure, cardiac index and mixed venous oxygen saturation.Data

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2017 EvidenceUpdates

17. Risk Assessment in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension. (PubMed)

Risk Assessment in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension. Current pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate, or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension in the French, Swedish, and COMPERA registries.To (...) investigate 3 abbreviated risk stratification methods for patients with mostly prevalent pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, in patients from the PATENT-1/-2 and CHEST-1/-2 studies of riociguat.Risk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.With all 3 methods, riociguat improved risk group/strata in patients with pulmonary arterial

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2019 European Respiratory Journal

18. Non-invasive screening using ventilatory gas analysis to distinguish between chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension. (PubMed)

Non-invasive screening using ventilatory gas analysis to distinguish between chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension. Clinical presentations associated with chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH) at rest are highly similar. Differentiating between CTEPH and PAH using non-invasive techniques remains challenging. Thus, we examined whether analysis of ventilatory gas in response to postural changes (...) can be useful as a non-invasive screening method for pulmonary hypertension (PH), and help differentiate CTEPH from PAH.We prospectively enrolled 90 patients with suspected PH and performed right heart catheterization, ventilation/perfusion scan and ventilatory gas analysis. Various pulmonary function parameters were examined in the supine and sitting postures, and postural changes were calculated (Δ(supine - sitting)).In total, 25 patients with newly diagnosed PAH, 40 patients with newly

2019 Respirology

19. Efficacy and Safety of the Use of Pulmonary Arterial Hypertension Pharmacotherapy in Patients with Pulmonary Hypertension Secondary to Left Heart Disease: A Systematic Review. (PubMed)

Efficacy and Safety of the Use of Pulmonary Arterial Hypertension Pharmacotherapy in Patients with Pulmonary Hypertension Secondary to Left Heart Disease: A Systematic Review. Pulmonary hypertension (PH) is often caused by left heart disease (LHD) such as heart failure (HF) or valvular heart disease. Historically, few randomized controlled trials have evaluated the off-label use of medications for treating pulmonary arterial hypertension (PAH) in patients with PH-LHD. However, multiple (...) and patients with isolated post-capillary PH due to HF. In addition, the use of bosentan or macitentan is not recommended in patients with combined post-capillary and pre-capillary PH due to HF, but sildenafil may be considered to improve pulmonary hemodynamics and exercise capacity in patients with combined post-capillary and pre-capillary PH due to HF. Riociguat 2 mg 3 times daily may also be considered to improve pulmonary hemodynamics in patients with combined post-capillary and pre-capillary PH due

2019 Pharmacotherapy

20. Transthoracic Pulmonary Artery Denervation for Pulmonary Arterial Hypertension. (PubMed)

Transthoracic Pulmonary Artery Denervation for Pulmonary Arterial Hypertension. Objective- Pulmonary arterial hypertension is characterized by progressive pulmonary vascular remodeling and persistently elevated mean pulmonary artery pressures and pulmonary vascular resistance. We aimed to investigate whether transthoracic pulmonary artery denervation (TPADN) attenuated pulmonary artery (PA) remodeling, improved right ventricular (RV) function, and affected underlying mechanisms. We also (...) explored the distributions of sympathetic nerves (SNs) around human PAs for clinical translation. Approach and Results- We identified numerous SNs in adipose and connective tissues around the main PA trunks and bifurcations in male Sprague Dawley rats, which were verified in samples from human heart transplant patients. Pulmonary arterial hypertensive rats were randomized into TPADN and sham groups. In the TPADN group, SNs around the PA trunk and bifurcation were completely and accurately removed under

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2019 Thrombosis and Vascular Biology

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