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Pulmonary Arterial Hypertension Management

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1. 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

2. Pulmonary arterial hypertension: pathogenesis and clinical management. (PubMed)

Pulmonary arterial hypertension: pathogenesis and clinical management. Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of pulmonary hypertension that primarily affects the pulmonary vasculature. In PAH, the pulmonary vasculature is dynamically obstructed by vasoconstriction, structurally obstructed by adverse vascular remodeling, and pathologically non-compliant as a result (...) , macrophages), and autoantibodies suggest that PAH is, in part, an autoimmune, inflammatory disease. Obstructive pulmonary vascular remodeling in PAH increases right ventricular afterload causing right ventricular hypertrophy. In some patients, maladaptive changes in the right ventricle, including ischemia and fibrosis, reduce right ventricular function and cause right ventricular failure. Patients with PAH have dyspnea, reduced exercise capacity, exertional syncope, and premature death from right

2018 BMJ

3. ESC/ESH Management of Arterial Hypertension

ESC/ESH Management of Arterial Hypertension We use cookies to enhance your experience on our website. By continuing to use our website, you are agreeing to our use of cookies. You can change your cookie settings at any time. 2018 ESC/ESH Guidelines for the management of arterial hypertension | European Heart Journal | Oxford Academic Search Account Menu Menu Navbar Search Filter Mobile Microsite Search Term Close search filter search input Article Navigation Close mobile search navigation (...) Article navigation 01 September 2018 Article Contents Article Navigation 2018 ESC/ESH Guidelines for the management of arterial hypertension Bryan Williams ESC Chairperson Corresponding authors. Bryan Williams, Institute of Cardiovascular Science, University College London, Maple House, 1st Floor, Suite A, 149 Tottenham Court Road, London W1T 7DN, UK, Tel: +44 (0) 20 3108 7907, E-mail: . Search for other works by this author on: Giuseppe Mancia ESH Chairperson Giuseppe Mancia, University of Milano

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2018 European Society of Cardiology

4. Pulmonary Arterial Hypertension Diagnosis, Treatment, and Clinical Management in the Contemporary Era (PubMed)

Pulmonary Arterial Hypertension Diagnosis, Treatment, and Clinical Management in the Contemporary Era Pulmonary arterial hypertension (PAH) is characterized by severe remodeling of the distal pulmonary arteries, increased pulmonary vascular resistance, and right ventricular dysfunction that promotes heart failure. Once regarded as largely untreatable, evidence-based decision making now guides clinical management of PAH and improves outcomes. However, misconceptions regarding the approach to PAH

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2016 JAMA cardiology

5. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies (PubMed)

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular (...) resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud's phenomenon

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2016 Open access rheumatology : research and reviews

6. Wireless pulmonary artery pressure monitoring with CardioMEMS HF System (St. Jude Medical) for management of chronic heart failure

pressure, and diabetes. HF is characterized by the inability of the heart to pump blood efficiently. Dysfunctional conditions of the heart, such as those associated with HF, can lead to pulmonary hypertension. Thus, monitoring changes in pulmonary artery pressure (PAP) may provide a way to monitor changes in HF and result in improved HF management. Description of Technology: The CardioMEMS (CM) HF System is a wireless implantable hemodynamic monitor (IHM) that measures PAP and heart rate in patients (...) Wireless pulmonary artery pressure monitoring with CardioMEMS HF System (St. Jude Medical) for management of chronic heart failure Wireless pulmonary artery pressure monitoring with CardioMEMS HF System (St. Jude Medical) for management of chronic heart failure Wireless pulmonary artery pressure monitoring with CardioMEMS HF System (St. Jude Medical) for management of chronic heart failure HAYES, Inc Record Status This is a bibliographic record of a published health technology assessment

2017 Health Technology Assessment (HTA) Database.

7. Implication of pulmonary-systemic flow information in the management of complex presentation of pulmonary arterial hypertension: exploring role of phase contrast MRI technique (PubMed)

Implication of pulmonary-systemic flow information in the management of complex presentation of pulmonary arterial hypertension: exploring role of phase contrast MRI technique 28516053 2018 11 13 2223-4292 7 2 2017 Apr Quantitative imaging in medicine and surgery Quant Imaging Med Surg Implication of pulmonary-systemic flow information in the management of complex presentation of pulmonary arterial hypertension: exploring role of phase contrast MRI technique. 276-280 10.21037/qims.2016.12.01

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2017 Quantitative imaging in medicine and surgery

8. Management dilemmas in pulmonary arterial hypertension associated with congenital heart disease (PubMed)

Management dilemmas in pulmonary arterial hypertension associated with congenital heart disease There are few randomised controlled data to guide management of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). In this clinical review, common areas of uncertainty in the management of PAH-CHD are identified, the literature is summarised and discussed and a suggested approach offered for each clinical dilemma.

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2018 Pulmonary circulation

9. Advanced therapies for the management of adults with pulmonary arterial hypertension due to congenital heart disease: a systematic review. (PubMed)

Advanced therapies for the management of adults with pulmonary arterial hypertension due to congenital heart disease: a systematic review. Pulmonary arterial hypertension (PAH) secondary to congenital heart disease (CHD) is the third most common cause of PAH, and it is becoming increasingly common as improvements in the management of CHD have led to increased life expectancy for these patients. The medical management of PAH due to CHD (PAH-CHD) is largely the same as what has been used (...) for the treatment of idiopathic PAH, though the body of literature supporting this management decision is very small. There are currently few studies available which specifically focus on the treatment of PAH-CHD. The purpose of this literature review is to compare the results of those studies that assessed the response to medical therapy among adults with PAH-CHD; studies were excluded if they focused on paediatric patients, did not include an assessment of 6 min walking distance or specifically assessed

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2018 Open heart

10. Extended-release oral treprostinil in the management of pulmonary arterial hypertension: clinical evidence and experience (PubMed)

Extended-release oral treprostinil in the management of pulmonary arterial hypertension: clinical evidence and experience Treprostinil diolamine is the first oral prostacyclin approved for the treatment of pulmonary arterial hypertension (PAH) to improve exercise capacity. Clinical studies have demonstrated modest benefit as monotherapy, whereas no difference in exercise capacity was observed with combination therapy. However, these trials were limited by subtherapeutic dosing owing

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2018 Therapeutic advances in respiratory disease

11. Use of Extracorporeal Membrane Oxygenation in Postpartum Management of a Patient with Pulmonary Arterial Hypertension (PubMed)

Use of Extracorporeal Membrane Oxygenation in Postpartum Management of a Patient with Pulmonary Arterial Hypertension Current guidelines do not recommend pregnancy in patients with pulmonary arterial hypertension (PAH). This is due to the associated high mortality, which both dissuades PAH patients from becoming pregnant and encourages termination of pregnancy due to high maternal mortality risk. As a result, there is a lack of data and, consequently, there are only general guidelines available (...) for management of pregnancy in PAH patients. Additionally, novel therapeutic strategies such as extracorporeal membrane oxygenation (ECMO), although used in the management of nonpregnant PAH patients as a bridge to lung transplantation, have not been used to treat cardiopulmonary collapse in pregnant PAH patients. In an attempt to bridge this paucity of data, we report the successful use of ECMO in resuscitation and management of a pregnant PAH patient who experienced cardiopulmonary collapse following

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2018 Case reports in pulmonology

12. Successful Management of Pregnancy in a Patient with Systemic Lupus Erythematosus-associated Pulmonary Arterial Hypertension (PubMed)

Successful Management of Pregnancy in a Patient with Systemic Lupus Erythematosus-associated Pulmonary Arterial Hypertension Pregnancy in women with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) remains a high risk. We successfully managed a pregnancy in a patient with SLE-PAH. A 31-year-old pregnant woman with SLE-PAH had worsening PAH and SLE flare-up during pregnancy and a sudden increase in pulmonary arterial pressure after delivery. SLE-PAH

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2018 Internal Medicine

13. Endothelin-receptor antagonists in the management of pulmonary arterial hypertension: where do we stand? (PubMed)

Endothelin-receptor antagonists in the management of pulmonary arterial hypertension: where do we stand? Pulmonary arterial hypertension, a disease largely neglected until a few decades ago, is presently the object of intense studies by several research teams. Despite considerable progress, pulmonary arterial hypertension remains a major clinical problem, because it is not always easy to diagnose, treat, and prevent. The disease was considered incurable until the late 1990s, when Epoprostenol (...) was introduced as the first tool against this illness. More recently, therapy for pulmonary arterial hypertension gained momentum after publication of the SERAPHIN and AMBITION trials, which also highlighted the importance of upfront therapy. This review also focuses on recent substudies from these trials and progress in drugs targeting the endothelin pathway. Future perspectives with regard to endothelin-receptor antagonists are also discussed.

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2018 Vascular health and risk management

14. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. (PubMed)

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Paediatric pulmonary arterial hypertension (PAH) shares common features of adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary (...) Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps. Several features of paediatric PAH including the prominence of neonatal PAH, especially in pre-term infants with developmental lung diseases, and novel genetic causes of paediatric PAH are highlighted. The use of cardiac catheterisation as a diagnostic modality and haemodynamic definitions of PAH, including acute vasoreactivity

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2018 European Respiratory Journal

15. 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 (...) the Grading of Recommendations Assessment, Development and Evaluation approach. Graded recommendations and ungraded consensus-based statements were developed and voted on using a modified Delphi technique to achieve consensus.Two new recommendations on combination therapy and two ungraded consensus-based statements on palliative care were developed. An evidence-based and consensus-driven treatment algorithm was created to guide the clinician through an organized approach to management, and to direct

2019 EvidenceUpdates

16. Low plasma stem cell factor combined with high transforming growth factor-α identifies high-risk patients in pulmonary arterial hypertension (PubMed)

Early and Long-Term Pulmonary Arterial Hypertension Disease Management) risk scores in PAH patients correlated to SCF/TGF-α levels (r=-0.50, p=0.0003). In conclusion, low plasma SCF combined with high TGF-α identifies high-risk PAH patients at baseline. Lower circulating SCF levels, which are associated with worse haemodynamics, may be related to the c-Kit accumulation previously observed in PAH. (...) Low plasma stem cell factor combined with high transforming growth factor-α identifies high-risk patients in pulmonary arterial hypertension In pulmonary arterial hypertension (PAH), severe vasoconstriction and remodelling of small pulmonary arteries result in high mortality. Receptor tyrosine kinases and their ligands, such as transforming growth factor (TGF)-α, modulate proliferation in PAH. Although the receptor tyrosine kinase c-Kit has been shown to be overexpressed in PAH, the expression

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2018 ERJ open research

17. Hypertension in pregnancy: diagnosis and management

: diagnosis and management (NG133) © NICE 2019. All rights reserved. Subject to Notice of rights (https://www.nice.org.uk/terms-and- conditions#notice-of-rights). Page 24 of 54umbilical artery doppler velocimetry. [2010] [2010] 1.6.7 If the results of all fetal monitoring are normal in women with pre-eclampsia or severe gestational hypertension, do not routinely repeat cardiotocography unless clinically indicated. [2010, amended 2019] [2010, amended 2019] 1.6.8 In women with pre-eclampsia or severe (...) Carry out an ultrasound for fetal growth and amniotic fluid volume assessment and umbilical artery doppler velocimetry starting at between 28 and 30 weeks (or at least 2 weeks before previous gestational age of onset if earlier than 28 weeks) and repeating 4 weeks later in women with previous: severe pre-eclampsia Hypertension in pregnancy: diagnosis and management (NG133) © NICE 2019. All rights reserved. Subject to Notice of rights (https://www.nice.org.uk/terms-and- conditions#notice-of-rights

2019 National Institute for Health and Clinical Excellence - Clinical Guidelines

18. The Prognostic Value of Follow-up Hemodynamic Variables After Initial Management in Pulmonary Arterial Hypertension. (PubMed)

The Prognostic Value of Follow-up Hemodynamic Variables After Initial Management in Pulmonary Arterial Hypertension. Hemodynamic variables such as cardiac index and right atrial pressure have consistently been associated with survival in pulmonary arterial hypertension (PAH) at the time of diagnosis. Recent studies have suggested that pulmonary arterial compliance may also predict prognosis in PAH. The prognostic importance of hemodynamic values achieved after treatment initiation is less well (...) established.Our objective was to evaluate the prognostic importance of clinical and hemodynamic variables during follow-up, including pulmonary arterial compliance, after initial management in PAH. We evaluated incident patients with idiopathic, drug- and toxin-induced, or heritable PAH enrolled in the French pulmonary hypertension registry between 2006 and 2016 who had a follow-up right-sided heart catheterization (RHC). The primary outcome was death or lung transplantation. We used stepwise Cox regression

2017 Circulation

19. Considerations for optimal management of patients with pulmonary arterial hypertension: a multi-stakeholder roundtable discussion. (PubMed)

Considerations for optimal management of patients with pulmonary arterial hypertension: a multi-stakeholder roundtable discussion. A roundtable panel of national and regional managed care decision makers and providers met to discuss pulmonary arterial hypertension (PAH) and strategies for management. As a rare, complex disease with high economic costs and potentially devastating outcomes, PAH necessitates that managed care providers balance optimal care with efficient use of healthcare (...) resources. PAH specialists are recognized by health plans as knowledgeable experts and integral partners in managing patients and resources. The diagnosis of PAH must be confirmed by a right heart catheterization. Available therapies are indicated almost exclusively for patients with PAH (riociguat is also indicated in chronic thromboembolic pulmonary hypertension) and target 1 of 3 pathways: endothelin receptor antagonists for the endothelin pathway; phosphodiesterase type-5 inhibitors and soluble

2017 American Journal Of Managed Care

20. The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension. (PubMed)

The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension. Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies (...) . A committee of physicians from nationally designated PH centres identified the 'most common' emergency clinical scenarios encountered in patients with PAH. Thereafter, a review of the literature was performed centred on these specified topics and a management approach was developed based on best available evidence and expert consensus. Management protocols were developed on the following PAH emergencies: chest pain (including myocardial ischaemia), right ventricular failure, arrhythmias, sepsis

2017 Thorax

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