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Pulmonary hypertension

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64 papers 0 to 25 followers
https://www.readbyqxmd.com/read/27872804/pulmonary-hypertension-secondary-to-pulmonary-veno-occlusive-disease-complicated-by-right-heart-failure-hypotension-and-acute-kidney-injury
#1
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
#2
REVIEW
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.
November 21, 2016: 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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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...
September 5, 2016: 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
#9
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
https://www.readbyqxmd.com/read/27613549/the-right-ventricle-interaction-with-the-pulmonary-circulation
#10
REVIEW
Michael R Pinsky
The primary role of the right ventricle (RV) is to deliver all the blood it receives per beat into the pulmonary circulation without causing right atrial pressure to rise. To the extent that it also does not impede left ventricular (LV) filling, cardiac output responsiveness to increased metabolic demand is optimized. Since cardiac output is a function of metabolic demand of the body, during stress and exercise states the flow to the RV can vary widely. Also, instantaneous venous return varies widely for a constant cardiac output as ventilatory efforts alter the dynamic pressure gradient for venous return...
September 10, 2016: Critical Care: the Official Journal of the Critical Care Forum
https://www.readbyqxmd.com/read/27615023/comparative-effectiveness-of-pharmacological-interventions-for-pulmonary-arterial-hypertension-a-systematic-review-and-network-meta-analysis
#11
Snigdha Jain, Rohan Khera, Saket Girotra, David Badesch, Zhen Wang, Mohammad Hassan Murad, Amy Blevins, Gregory A Schmidt, Siddharth Singh, Alicia K Gerke
BACKGROUND: We conducted a systematic review and network meta-analysis to examine comparative efficacy and tolerability of pharmacological interventions for pulmonary arterial hypertension (PAH). METHODS: MEDLINE, Cochrane register, EMBASE, CINAHL and clinicaltrials.gov were searched (January 1, 1990 - March 3, 2016). Randomized controlled trials (RCTs) of approved pharmacological agents - endothelin receptor antagonists (ERA), phosphodiesterase inhibitors (PDE5i), oral/inhaled and intravenous/subcutaneous prostanoids, riociguat, and selexipag, alone or in combination for pulmonary arterial hypertension (PAH) reporting at least one efficacy outcome were selected...
September 8, 2016: Chest
https://www.readbyqxmd.com/read/27571000/chronic-thromboembolic-pulmonary-hypertension-the-journey-from-a-curiosity-to-a-cure
#12
William R Auger, Jess Mandel, Joanna Pepke-Zaba
Chronic thromboembolic pulmonary hypertension is a progressive and life-threatening condition that is believed to be related to inadequate dissolution of acute pulmonary thromboemboli, followed by fibrotic organization. Without appropriate treatment, progressive pulmonary hypertension, right ventricular failure, and death may occur. While the disorder was initially described nearly a century ago in autopsy studies, antemortem diagnosis became feasible with the advent of cardiac catheterization and angiography techniques in the 1940s, while surgical thromboendarterectomy was not attempted until the 1960s...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27571001/chronic-thromboembolic-pulmonary-hypertension-epidemiology-and-risk-factors
#13
Marion Delcroix, Kim Kerr, Peter Fedullo
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism. As for most rare diseases, epidemiologic data are scarce, but recent registries suggest an incidence of at least 5 per million inhabitants per year. A history of massive or recurrent acute pulmonary embolism is observed in most patients with CTEPH, but the proportion of patients who develop CTEPH after acute pulmonary embolism is a matter of debate, further complicated by the possible misdiagnosis of CTEPH as acute pulmonary embolism...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27571002/from-acute-to-chronic-thromboembolic-disease
#14
Timothy Fernandes, Benjamin Planquette, Olivier Sanchez, Timothy Morris
After achievement of adequate anticoagulation, the natural history of acute pulmonary emboli ranges from near total resolution of vascular perfusion to long-term persistence of hemodynamically consequential residual perfusion defects. The persistence of perfusion defects is necessary, but not sufficient, for the development of chronic thromboembolic pulmonary hypertension (CTEPH). Approximately 30% of patients have persistent defects after 6 months of anticoagulation, but only 10% of those with persistent defects subsequently develop CTEPH...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27571004/diagnostic-evaluation-of-chronic-thromboembolic-pulmonary-hypertension
#15
Deepa Gopalan, Daniel Blanchard, William R Auger
Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 25 mm Hg. Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as pulmonary hypertension in the presence of an organized thrombus within the pulmonary vascular bed that persists at least 3 months after the onset of anticoagulant therapy. Because CTEPH is potentially curable by surgical endarterectomy, correct identification of patients with this form of pulmonary hypertension and an accurate assessment of surgical candidacy are essential to provide optimal care...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27571005/pulmonary-endarterectomy-patient-selection-technical-challenges-and-outcomes
#16
Michael Madani, Eckhard Mayer, Elie Fadel, David P Jenkins
In chronic thromboembolic pulmonary hypertension, thromboemboli do not undergo resolution but instead become highly organized and fibrotic, resulting in obstruction of segments of the pulmonary vascular tree. Progressive pulmonary hypertension ensues, with substantial associated morbidity and mortality. Despite advances in medical therapy for some types of pulmonary hypertension, surgical pulmonary endarterectomy, also referred to as pulmonary thromboendarterectomy, remains the only potentially curative option for patients with chronic thromboembolic pulmonary hypertension...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27571006/medical-therapy-in-chronic-thromboembolic-pulmonary-hypertension
#17
Joanna Pepke-Zaba, Xavier Jais, Richard Channick
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening condition resulting from unresolved thromboembolic obstructions. Pulmonary endarterectomy surgery is currently the standard of treatment, as it is potentially curative; however, not all cases are amenable to surgical intervention due to distal distribution of the organized thromboembolic material or the presence of comorbidities. Up to one-third of patients have persistent or recurrent pulmonary hypertension after pulmonary endarterectomy...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27571007/future-directions-in-chronic-thromboembolic-pulmonary-hypertension-disease-at-a-crossroads
#18
Nick H Kim, Gérald Simonneau
In looking toward the future of chronic thromboembolic pulmonary hypertension, the following four clinical questions deserve careful consideration: (1) What is inoperable disease, and how is it defined? (2) Is there a role for targeted medical therapy in technically operable, proximal disease? (3) Where does balloon pulmonary angioplasty fit into the treatment algorithm? (4) How should we approach patients with symptomatic chronic pulmonary thromboembolism without pulmonary hypertension? The search for these answers will continue to propel this field forward...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27575357/clinical-relevance-of-fluid-challenge-in-patients-evaluated-for-pulmonary-hypertension
#19
Michele D'Alto, Emanuele Romeo, Paola Argiento, Yoshiki Motoji, Anna Correra, Giovanni Maria Di Marco, Agostino Mattera Iacono, Rosaria Barracano, Antonello D'Andrea, Gaetano Rea, Berardo Sarubbi, Maria Giovanna Russo, Robert Naeije
BACKGROUND: A fluid challenge may help to the differential diagnosis between pre- and post-capillary pulmonary hypertension (PH). However the test is still in need of standardization and better defined clinical relevance. METHODS AND RESULTS: Two-hundred-twelve patients referred for PH underwent a right heart catheterization with measurements before and after rapid infusion of 7 ml/kg of saline. PH was defined by a mean pulmonary artery pressure (mPAP) ≥ 25 mmHg and post-capillary PH by a wedged PAP (PAWP) > 15 mmHg...
August 26, 2016: Chest
https://www.readbyqxmd.com/read/27554299/pulmonary-hypertension-in-diffuse-parenchymal-lung-diseases
#20
Oksana A Shlobin, A Whitney Brown, Steven D Nathan
Pulmonary hypertension (PH) can be triggered by any number of disease processes that result in increased pulmonary vascular resistance. Although historically associated with idiopathic pulmonary arterial hypertension (iPAH), the majority of patients with PH do not have the idiopathic subtype, but rather PH associated with another underlying diagnosis, such as left heart or lung disease. The World Health Organization (WHO) classification of PH helps conceptualize the different categories based on presumed etiology...
August 20, 2016: Chest
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