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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

4. 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

5. 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

6. 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

7. 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

8. 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.

9. 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.

10. 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

11. 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

12. 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

13. Pulmonary Artery Size in Interstitial Lung Disease and Pulmonary Hypertension: Association with Interstitial Lung Disease Severity and Diagnostic Utility (PubMed)

Pulmonary Artery Size in Interstitial Lung Disease and Pulmonary Hypertension: Association with Interstitial Lung Disease Severity and Diagnostic Utility It is postulated that ILD causes PA dilatation independent of the presence of pulmonary hypertension (PH), so the use of PA size to screen for PH is not recommended. The aims of this study were to investigate the association of PA size with the presence and severity of ILD and to assess the diagnostic accuracy of PA size for detecting (...) PH.Incident patients referred to a tertiary PH centre underwent baseline thoracic CT, MRI and right heart catheterisation (RHC). Pulmonary artery diameter was measured on CT pulmonary angiography and pulmonary arterial areas on MRI. A thoracic radiologist scored the severity of ILD on CT from 0 to 4, 0 = absent, 1 = 1-25%, 2 = 26-50%, 3 = 51-75%, and 4 = 76-100% extent of involvement. Receiver operating characteristic analysis and linear regression were employed to assess diagnostic accuracy

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2018 Frontiers in cardiovascular medicine

14. Isolating pulmonary microvascular endothelial cells ex vivo: Implications for pulmonary arterial hypertension, and a caution on the use of commercial biomaterials. (PubMed)

Isolating pulmonary microvascular endothelial cells ex vivo: Implications for pulmonary arterial hypertension, and a caution on the use of commercial biomaterials. Transcriptomic analysis of pulmonary microvascular endothelial cells from experimental models offers insight into pulmonary arterial hypertension (PAH) pathobiology. However, culturing may alter the molecular profile of endothelial cells prior to analysis, limiting the translational relevance of results. Here we present a novel (...) and validated method for isolating RNA from pulmonary microvascular endothelial cells (PMVECs) ex vivo that does not require cell culturing. Initially, presumed rat PMVECs were isolated from rat peripheral lung tissue using tissue dissociation and enzymatic digestion, and cells were cultured until confluence to assess endothelial marker expression. Anti-CD31, anti-von Willebrand Factor, and anti-α-smooth muscle actin immunocytochemistry/immunofluorescence signal was detected in presumed rat PMVECs, but also

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2019 PLoS ONE

15. Pulmonary artery hypertension following coronary artery bypass grafting: a case report (PubMed)

Pulmonary artery hypertension following coronary artery bypass grafting: a case report Post-operative pulmonary complications in coronary artery bypass grafting (CABG) surgery are mostly reversible. We report a patient who developed pulmonary arterial hypertension (PAH) post-CABG and did not have pulmonary hypertension prior to surgery. PAH Group 1 was diagnosed after right and left heart catheterization. To the best of our knowledge, this is the only reported case of a patient developing PAH

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2018 ESC heart failure

16. 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 Pulmonary arterial hypertension (PAH) carries a poor prognosis if not promptly diagnosed and appropriately treated. The development and approval of 14 medications over the last several decades have led to a rapidly evolving approach to therapy, and have necessitated periodic updating of evidence-based treatment guidelines. This guideline statement, which now includes a visual algorithm

2019 EvidenceUpdates

17. Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension. (PubMed)

Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension. Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality rate despite the progress achieved with pulmonary arterial vasodilator therapies.We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease (...) phenotype when compared with idiopathic pulmonary arterial hypertension (IPAH) on the basis of progression of echocardiographic right ventricular (RV) dysfunction.Retrospective analysis of echocardiographic data in 13 patients with SSc-PAH and 11 patients with IPAH was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy. All patients had right heart catheterization-confirmed pulmonary arterial hypertension as well as complete baseline

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2017 Annals of the American Thoracic Society

18. Bosentan therapy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: A systemic review and meta-analysis.

Bosentan therapy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: A systemic review and meta-analysis. Bosentan therapy has been recommended for pulmonary arterial hypertension (PAH) and might be beneficial for chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to evaluate the specific effects of bosentan for PAH and CTEPH.We performed a systemic review and meta-analysis of randomized controlled trials (RCTs), comparing efficacy and safety (...) of bosentan treatment for PAH and CTEPH through major biomedical database.A total of 10 RCTs including 1185 patients were enrolled. For PAH patients, bosentan prolonged 6-minute walk distance with a weighted mean difference of 35.7 m, reduced mean pulmonary arterial pressure by 5.7 mm Hg, increased cardiac index by 0.4 L/min/m2 , reduced pulmonary vascular resistance by 305.1 dyn·s/cm5 , prevented functional class from deterioration and reduced clinical worsening as compared with placebo. For CTEPH

2018 The clinical respiratory journal

19. Coping, social support and information in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A 2-year retrospective cohort study (PubMed)

Coping, social support and information in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A 2-year retrospective cohort study Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are severe diseases with complicated treatment that need care at specialist clinics. The aim was to investigate changes in the patients' perceptions on coping, social support and received information when attending a newly started nurse (...) -coordinated pulmonary arterial hypertension-outpatient clinic.The present study was a descriptive, questionnaire-based cohort study including 42 adult patients. To evaluate coping, the Pearlin Mastery Scale was used. Social support, information and health-related quality of life were measured using Social Network and Support Scale, QLQ-INFO25 and the EQ-5D.Attending the pulmonary arterial hypertension-outpatient clinic increased coping ability (Mastery Scale) significantly (baseline 16.0 ± 3.3 points vs 2

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2018 SAGE open medicine

20. Health-related quality of life, treatment adherence and psychosocial support in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. (PubMed)

Health-related quality of life, treatment adherence and psychosocial support in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) share similar quality of life impairment. The aim of the present study was to investigate health-related quality of life (HRQoL) and its relation to the perception of treatment and psychosocial support among PAH and CTEPH (...) patients. All adult PAH or CTEPH patients in the Swedish Pulmonary Arterial Hypertension Register were invited to participate in a national cohort survey. The survey included the EuroQol 5-dimensions (EQ-5D) instrument that measures an individual's HRQoL; the Beliefs about Medicines Questionnaire-Specific Scale that assesses the perception of PAH-specific treatment; the Mastery scale that evaluates the feeling of control and ability to cope with the disease; and the Social Network and Support Scale

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2018 Chronic respiratory disease

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