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Pulmonary hypertension

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https://www.readbyqxmd.com/read/28096285/epoprostenol-and-pulmonary-arterial-hypertension-20%C3%A2-years-of-clinical-experience
#1
REVIEW
Olivier Sitbon, Anton Vonk Noordegraaf
Epoprostenol was the first therapy to be approved for the treatment of pulmonary arterial hypertension (PAH). In the 20 years since the introduction of this prostacyclin analogue, the outlook for patients with PAH has improved, with survival rates now double those from the era before the development of disease-specific treatments. Today, there are a large amount of data on the clinical role of prostacyclin treatments and a body of evidence attesting the efficacy of epoprostenol in improving exercise capacity, key haemodynamic parameters and PAH symptoms, as well as in reducing mortality...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28223154/idiopathic-and-systemic-sclerosis-associated-pulmonary-arterial-hypertension-a-comparison-of-demographic-haemodynamic-and-magnetic-resonance-imaging-characteristics-and-outcomes
#2
Sheila Ramjug, Nehal Hussain, Judith Hurdman, Catherine Billings, Athanasios Charampopoulos, Charlie A Elliot, David G Kiely, Ian Sabroe, Smitha Rajaram, Andrew J Swift, Robin Condliffe
BACKGROUND: Previous studies have identified survival in systemic sclerosis (SSc)-associated pulmonary arterial hypertension (SSc-PAH) to be worse than in idiopathic pulmonary arterial hypertension (IPAH). We investigated differences between these conditions by comparing demographic, haemodynamic and radiological characteristics and outcomes in a large cohort of incident patients. METHODS: 651 patients diagnosed with IPAH or SSc-associated pre-capillary pulmonary hypertension were included...
February 18, 2017: Chest
https://www.readbyqxmd.com/read/28026153/clinical-and-hemodynamic-characteristics-of-pulmonary-hypertension-associated-with-interstitial-lung-disease-in-china
#3
Lan Wang, Qin-Hua Zhao, Bigyan Pudasaini, Rong Jiang, Su-Gang Gong, Jing He, Ping Yuan, Jin-Ming Liu
BACKGROUND: Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD). The aim of the present study was to investigate characteristics and prognosis of ILD associated PH in a predominantly Chinese patient cohort. METHODS: Consecutive patients with ILD associated PH, confirmed by right heart catheterization at shanghai pulmonary hospital were included in the study. Characteristics at baseline and during follow-up, as well as survival, were analyzed...
December 27, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28052583/pulmonary-hypertension-in-advanced-lung-diseases-echocardiography-as-an-important-part-of-patient-evaluation-for-lung-transplantation
#4
Jolanta Nowak, Bartosz Hudzik, Dariusz Jastrze Bski, Jacek T Niedziela, Piotr Rozentryt, Jacek Wojarski, Marek Ochman, Wojciech Karolak, Sławomir Żegleń, Marek Gierlotka, Mariusz Gąsior
INTRODUCTION: Pulmonary hypertension (PH) is common complication in advanced lung disease. Echocardiography provides additional information and may be useful to assess PH probability. OBJECTIVES: The usefulness of combination of well-known echocardiographic parameters in detecting PH in patients with advanced lung disease referred for lung transplantation was evaluated. METHODS: The study population consisted of 37 consecutive patients with idiopathic pulmonary fibrosis (IPF), 20 patients with chronic obstructive pulmonary disease (COPD), and 8 patients with other interstitial lung diseases...
January 4, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27903659/pulmonary-hypertension-the-importance-of-correctly-diagnosing-the-cause
#5
Sanjay Mehta, Jean-Luc Vachiéry
Pulmonary hypertension (PH) is a complex condition that can occur as a result of a wide range of disorders, including left heart disease, lung disease and chronic pulmonary thromboembolism. Contemporary PH patients are older and frequently have a multitude of comorbidities that may contribute to or simply coincide with their PH. Identifying the cause of PH in these complicated patients can be challenging but is essential, given that the aetiology of the disease has a significant impact on the management options available...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27903660/future-perspectives-in-pulmonary-arterial-hypertension
#6
REVIEW
Gérald Simonneau, Marius M Hoeper, Vallerie McLaughlin, Lewis Rubin, Nazzareno Galiè
While there have been advances in the field of pulmonary arterial hypertension (PAH), disease management remains suboptimal for many patients. The development of novel treatments and strategies can provide opportunities to target other mechanisms that play a role in the complex pathobiology of PAH outside of the three main pathophysiological pathways. In this review, we highlight some of the potential PAH therapies or techniques that are being, or have been, investigated in phase II clinical trials. This review also discusses potential points for consideration in the development of novel therapies that target putative disease mediators or modifiers...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27903661/risk-assessment-in-pulmonary-arterial-hypertension
#7
REVIEW
Amresh Raina, Marc Humbert
Regular patient assessment is essential for the management of chronic diseases, such as pulmonary arterial hypertension (PAH). Comprehensive patient assessment and risk stratification in PAH are important to guide treatment decisions and to monitor disease progression as well as patients' response to treatment. Approaches for assessing risk in PAH patients include the use of risk variables, as recommended in the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines, and the application of risk equations and scores, such as the French registry risk equation and the REVEAL registry risk score...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27903662/patient-engagement-and-self-management-in-pulmonary-arterial-hypertension
#8
REVIEW
Jytte Graarup, Pisana Ferrari, Luke S Howard
Improved care in pulmonary arterial hypertension has led to increased longevity for patients, with a paralleled evolution in the nature of their needs. There is more focus on the impact of the disease on their day-to-day activities and quality of life, and a holistic approach is coming to the front of pulmonary arterial hypertension management, which places the patient at the centre of their own healthcare. Patients are thus becoming more proactive, involved and engaged in their self-care, and this engagement is an important factor if patient outcomes are to improve...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27903663/beyond-a-single-pathway-combination-therapy-in-pulmonary-arterial-hypertension
#9
REVIEW
Olivier Sitbon, Sean Gaine
There is a strong rationale for combining therapies to simultaneously target three of the key pathways implicated in the pathogenesis of pulmonary arterial hypertension (PAH). Evidence to support this strategy is growing, and a number of studies have demonstrated that combination therapy, administered as either a sequential or an initial regimen, can improve long-term outcomes in PAH. Dual combination therapy with a phosphodiesterase-5 inhibitor and an endothelin receptor antagonist is the most widely utilised combination regimen...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27903664/practical-considerations-for-therapies-targeting-the-prostacyclin-pathway
#10
REVIEW
Harrison W Farber, Wendy Gin-Sing
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. We discuss here a number of measures that can be taken to support patients and healthcare professionals in order to address the complexities of using these therapies and to encourage compliance...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27903665/pregnancy-in-pulmonary-arterial-hypertension
#11
REVIEW
Karen M Olsson, Richard Channick
Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant...
December 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27872804/pulmonary-hypertension-secondary-to-pulmonary-veno-occlusive-disease-complicated-by-right-heart-failure-hypotension-and-acute-kidney-injury
#12
Nima Golzy, Stuti Fernandes, Justin Sharim, Rikin Tank, Henry D Tazelaar, Howard E Epstein, Victor Tapson, Antoine Hage
Pulmonary veno-occlusive disease (PVOD) is rare condition which can lead to severe pulmonary hypertension, right ventricular dysfunction, and cardiopulmonary failure. The diagnosis of PVOD can be challenging due to its nonspecific symptoms and its similarity to idiopathic pulmonary arterial hypertension and interstitial lung disease in terms of diagnostic findings. This case describes a 57 year old female patient who presented with a 5-month history of progressive dyspnea on exertion and nonproductive cough...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27872176/the-evolving-landscape-of-combination-therapy-for-pulmonary-arterial-hypertension
#13
Matthew Griffin, Terence K Trow
Pulmonary arterial hypertension (PAH) is a progressively fatal disease, and the goal in treatment is to prevent disease progression. The standard of care often involves medications from multiple therapeutic classes, and there has been significant interest both in the choice of agent as well as the timing of initiation. There is a growing body of support for starting multiple medications at the time of diagnosis, or 'upfront ', rather than using sequential addition to prevent clinical deterioration.
February 2017: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/27784026/safety-tolerability-and-clinical-effects-of-a-rapid-dose-titration-of-subcutaneous-treprostinil-therapy-in-pulmonary-arterial-hypertension-a-prospective-multi-centre-trial
#14
Ekkehard Grünig, Nicola Benjamin, Tobias J Lange, Ulrich Krueger, Hans Klose, Claus Neurohr, Heinrike Wilkens, Michael Halank, Hans-Jürgen Seyfarth, Matthias Held, Andrew Traube, Michelle Pernow, E Robert Grover, Benjamin Egenlauf, Felix Gerhardt, Thomas Viethen, Stephan Rosenkranz
BACKGROUND: Subcutaneous treprostinil has dose-dependent beneficial effects in patients with severe pulmonary arterial hypertension, but adverse effects like infusion site pain can lead to treatment discontinuation. OBJECTIVES: The objective of this study was to evaluate safety, tolerability and clinical effects of a rapid up-titration dosing regimen of subcutaneous treprostinil using proactive infusion site pain management. METHODS: Effects of rapid up-titration dosing regimen on tolerability and clinical parameters were evaluated in this 16-week, open-label multi-centre study...
2016: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/27745818/initial-combination-therapy-with-ambrisentan-and-tadalafil-and-mortality-in-patients-with-pulmonary-arterial-hypertension-a-secondary-analysis-of-the-results-from-the-randomised-controlled-ambition-study
#15
Marius M Hoeper, Vallerie V McLaughlin, Joan Albert Barberá, Adaani E Frost, Hossein-Ardeschir Ghofrani, Andrew J Peacock, Gérald Simonneau, Stephan Rosenkranz, Ronald J Oudiz, R James White, Karen L Miller, Jonathan Langley, Julia H N Harris, Christiana Blair, Lewis J Rubin, Jean-Luc Vachiery
BACKGROUND: In treatment-naive patients with pulmonary arterial hypertension, initial combination therapy with ambrisentan and tadalafil reduces the risk of clinical failure events compared with monotherapy. We did this secondary analysis to further investigate the effect of combination therapy on survival. METHODS: We analysed survival data from the modified intention-to-treat population of the Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) trial...
October 10, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27746200/effect-of-balloon-pulmonary-angioplasty-on-respiratory-function-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#16
Mina Akizuki, Naoki Serizawa, Atsuko Ueno, Taku Adachi, Nobuhisa Hagiwara
RATIONALE: Balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH) improves hemodynamics and exercise capacity. However, its effect on respiratory function is unclear. OBJECTIVES: To investigate the effect of BPA on respiratory function. METHODS: We enrolled inoperable CTEPH who underwent BPA primarily in lower lobe arteries (first series) and upper and middle lobe arteries (second series). We compared changes in hemodynamics and respiratory function between different BPA fields...
October 13, 2016: Chest
https://www.readbyqxmd.com/read/27733022/long-term-outcomes-of-surgery-for-chronic-thromboembolic-pulmonary-hypertension-compared-with-medical-therapy-at-a-single-korean-center
#17
Soo Han Kim, Jae Won Lee, Jung-Min Ahn, Dae-Hee Kim, Jong-Min Song, Sang-Do Lee, Jae Seung Lee
Background/Aims: Pulmonary endarterectomy (PEA) is the gold standard for treating chronic thromboembolic pulmonary hypertension (CTEPH) in Western countries. The aim of this study was to investigate the long-term outcomes of performing PEA on CTEPH patients in comparison with medical therapy at a single Korean center. Methods: This retrospective study included 88 CTEPH patients. These patients were classified into the PEA group (n = 37) or non-PEA group (i.e., medical therapy; n = 51)...
October 13, 2016: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/27711018/liver-transplantation-and-pulmonary-hypertension-pathophysiology-and-management-strategies
#18
Michael Ramsay
PURPOSE OF REVIEW: Increases in pulmonary artery pressure are seen in 20% of patients with liver cirrhosis due to a high cardiac output state together with circulatory volume overload. Some patients develop true pulmonary arterial hypertension with pathological changes in the pulmonary vasculature, an elevated pulmonary vascular resistance, and an increased transpulmonary gradient. Survival of patients with portopulmonary hypertension is poor once it becomes moderate to severe, whether or not liver transplant is undertaken...
June 2007: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/27595643/a-review-of-therapeutic-agents-for-the-management-of-pulmonary-arterial-hypertension
#19
Stella S Hahn, Mina Makaryus, Arunabh Talwar, Mangala Narasimhan, Gulrukh Zaidi
Pulmonary arterial hypertension (PAH) is an uncommon, progressive and life threatening disease characterized by a proliferative vasculopathy of the small muscular pulmonary arterioles resulting in elevated pulmonary vascular resistance and eventually right ventricular failure. An increasing understanding of the pathobiology of PAH and its natural history has led to the development of numerous targeted therapies. Despite these advances there is significant progression of disease and the survival rate remains low...
January 2017: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/27581344/effects-of-balloon-pulmonary-angioplasty-on-oxygenation-in-patients-with-chronic-thromboembolic-pulmonary-hypertension%C3%A3-importance-of-intrapulmonary-shunt
#20
Tatsuo Aoki, Koichiro Sugimura, Kotaro Nochioka, Masanobu Miura, Shunsuke Tatebe, Saori Yamamoto, Nobuhiro Yaoita, Hideaki Suzuki, Haruka Sato, Katsuya Kozu, Satoshi Miyata, Kimio Satoh, Hiroaki Shimokawa
BACKGROUND: Although balloon pulmonary angioplasty (BPA) improves the hemodynamics and prognosis of patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), the mechanisms of improvement in oxygenation remain to be elucidated. METHODS AND RESULTS: From August 2013 to May 2015, we performed a total of 113 BPA procedures in 24 patients with inoperable CTEPH (mean 4.7 procedures per patient). Median age was 70 [60, 74] years and 18 were female (75%)...
September 23, 2016: Circulation Journal: Official Journal of the Japanese Circulation Society
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