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Pulmonary hypertension treatment

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https://www.readbyqxmd.com/read/28822963/%C3%AE-1a-subtype-adrenergic-agonist-therapy-for-failing-right-ventricle
#1
Patrick M Cowley, Guan-Ying Wang, Sunil K Joshi, Philip M Swigart, David H Lovett, Paul C Simpson, Anthony J Baker
Failure of the right ventricle (RV) is a serious disease with a poor prognosis and limited treatment options. Signaling by α1-adrenergic receptors (α1-ARs), in particular the α1A-subtype, mediate cardioprotective effects in multiple heart failure models. Recent studies show that chronic treatment with the α1A-subtype agonist A61603 improves function and survival in a model of LV failure. The goal of this study was to determine if chronic A61603 treatment is beneficial in a RV failure model. We used tracheal instillation of the fibrogenic antibiotic bleomycin in mice to induce pulmonary fibrosis, pulmonary hypertension, and RV failure within 2 wk...
August 19, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28820870/controversies-in-the-identification-and-management-of-acute-pulmonary-hypertension-in-preterm-neonates
#2
REVIEW
Regan E Giesinger, Kiran More, Jodie Odame, Amish Jain, Robert P Jankov, Patrick J McNamara
It is increasingly recognized that the abnormal physiologic consequences of pulmonary hypertension (PH) may contribute to poor cardiopulmonary health in premature babies. Conflicting literature has led to clinical uncertainty, pathological misinterpretation and variability in treatment approaches among practitioners. There are several disorders with overlapping and interrelated presentations and other disorders with a similar clinical phenotype but diverse pathophysiological contributors. In this review, we provide a diagnostic approach for acute HRF in the preterm neonate, outline the pathophysiological conditions that may present as acute PH and discuss the implications of high pulmonary vascular resistance (PVR) on the cardiovascular system...
August 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28819824/-update-pulmonary-arterial-hypertension-definitions-diagnosis-therapy
#3
N Sommer, M J Richter, K Tello, F Grimminger, W Seeger, H A Ghofrani, H Gall
The term pulmonary arterial hypertension comprises a group of pulmonary vascular diseases of different etiologies that are characterized by similar precapillary vascular remodeling processes and result in exertional dyspnea and right heart insufficiency. The specific pharmacological treatment approach considers the risk of mortality and phenotypical properties and includes treatment with phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostanoids, as well as with more novel substances, such as a soluble guanylyl cyclase stimulator and an oral prostacyclin receptor agonist...
August 17, 2017: Der Internist
https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#4
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28817980/pathways-to-pulmonary-hypertension-in-sickle-cell-disease-the-search-for-prevention-and-early-intervention
#5
Natalie R Shilo, Claudia R Morris
Pulmonary hypertension (PH) develops in a significant number of patients with sickle cell disease (SCD), resulting in increased morbidity and mortality. This review focuses on PH pathophysiology, risk stratification, and new recommendations for screening and treatment for patients with SCD. Areas covered: An extensive PubMed literature search was performed. While the pathophysiology of PH in SCD is yet to be fully deciphered, it is known that the etiology is multifactorial; hemolysis, hypercoagulability, hypoxemia, ischemic-reperfusion injury, oxidative stress, and genetic susceptibility all contribute in varying degrees to endothelial dysfunction...
August 18, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28817465/new-insights-into-the-recognition-classification-and-management-of-systemic-sclerosis-associated-pulmonary-hypertension
#6
Christopher J Mullin, Stephen C Mathai
PURPOSE OF REVIEW: Pulmonary hypertension is a common complication of systemic sclerosis (SSc), and remains a leading cause of morbidity and mortality. We will review recent developments in the recognition, classification and treatment of pulmonary hypertension in SSc. RECENT FINDINGS: Advances in screening for pulmonary arterial hypertension (PAH) and use of exercise haemodynamics may help to identify pulmonary vascular disease earlier in SSc. Recent studies have led to changes in recommendations for adjunct therapy and selection of pulmonary vasodilators for the treatment of SSc-associated PAH...
August 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28816096/management-of-severe-pulmonary-hemorrhage-in-a-neonate-on-veno-arterial-ecmo-by-the-temporary-clamping-of-the-endotracheal-tube-a-case-report
#7
Vaclav Vobruba, Tomas Grus, Frantisek Mlejnsky, Jan Belohlavek, Jan Hridel, Lukas Lambert
Severe pulmonary hemorrhage in the newborn is an infrequent, but life-threatening, event. A newborn with persistent pulmonary hypertension and a large persistent ductus arteriosus and open foramen ovale presented with hypoxemia and progressive right heart failure shortly after birth, requiring veno-arterial extracorporeal membrane oxygenation (ECMO) support. Twenty minutes after the initiation of ECMO, the patient developed severe pulmonary hemorrhage refractory to conventional treatment. As a last resort, the endotracheal tube was clamped...
August 1, 2017: Perfusion
https://www.readbyqxmd.com/read/28814664/pulmonary-arterial-hypertension-treatment-with-carvedilol-for-heart-failure-a-randomized-controlled-trial
#8
Samar Farha, Didem Saygin, Margaret M Park, Hoi I Cheong, Kewal Asosingh, Suzy Aa Comhair, Olivia R Stephens, Emir C Roach, Jacqueline Sharp, Kristin B Highland, Frank P DiFilippo, Donald R Neumann, W H Wilson Tang, Serpil C Erzurum
BACKGROUND: Right-sided heart failure is the leading cause of death in pulmonary arterial hypertension (PAH). Similar to left heart failure, sympathetic overactivation and β-adrenoreceptor (βAR) abnormalities are found in PAH. Based on successful therapy of left heart failure with β-blockade, the safety and benefits of the nonselective β-blocker/vasodilator carvedilol were evaluated in PAH. METHODS: PAH Treatment with Carvedilol for Heart Failure (PAHTCH) is a single-center, double-blind, randomized, controlled trial...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28814429/nintedanib-macrophage-activation-and-ameliorates-vascular-and-fibrotic-manifestations-in-the-fra2-mouse-model-of-systemic-sclerosis
#9
Jingang Huang, Christiane Maier, Yun Zhang, Alina Soare, Clara Dees, Christian Beyer, Ulrike Harre, Chih-Wei Chen, Oliver Distler, Georg Schett, Lutz Wollin, Jörg H W Distler
BACKGROUND: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc). METHODS: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed...
August 16, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28814329/severe-persistent-pulmonary-hypertension-of-the-newborn-and-dysmorphic-features-in-neonate-with-a-deletion-involving-twist1-and-phf14-a-case-report
#10
Carina Schinagl, Guro Reinholt Melum, Olaug Kristin Rødningen, Kathrine Bjørgo, Jannicke Hanne Andresen
BACKGROUND: Persistent pulmonary hypertension is a well-known disease of the newborn that in most cases responds well to treatment with nitric oxide and treatment of any underlying causes. Genetic causes of persistent pulmonary hypertension of the newborn are rare. The TWIST1 gene is involved in morphogenetics, and deletions are known to cause Saethre-Chotzen syndrome. Deletions of PHF14 have never been reported in neonates, but animal studies have shown a link between severe defects in lung development and deletions of this gene...
August 17, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28813774/pulmonary-embolism-caused-by-popliteal-vein-aneurysm-a-case-report
#11
Kevin Marquez, Kalyan Chakravarthy Potu, Chad Laurich, Randall Lamfers
In this case report, we describe an unusual episode of bilateral submassive pulmonary embolism (PE) caused by a popliteal vein aneurysm (PVA). The development of PE stems from many risk factors including obesity (BMI³ 30 kg/m2), hypertension, cigarette smoking (greater than 25 cigarettes per day), increasing age, surgery, immobility, malignancy, and inherited thrombophilia. A PVA is a rare but significant cause of PE. A 28-year-old male presented to the emergency department with progressive shortness of breath...
March 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28809956/natural-reversal-of-pulmonary-vascular-remodeling-and-right-ventricular-remodeling-in-su5416-hypoxia-treated-sprague-dawley-rats
#12
Makhosazane Zungu-Edmondson, Nataliia V Shults, Oleksiy Melnyk, Yuichiro J Suzuki
AIMS: Pulmonary arterial hypertension (PAH) is a lethal disease and improved therapeutic strategies are needed. Increased pulmonary arterial pressure, due to vasoconstriction and vascular remodeling, causes right ventricle (RV) failure and death in patients. The treatment of Sprague-Dawley rats with SU5416 injection and exposure to chronic hypoxia for three weeks followed by maintenance in normoxia promote progressive and severe PAH with pathologic features that resemble human PAH. At 5-17 weeks after the SU5416 injection, PAH is developed with pulmonary vascular remodeling as well as RV hypertrophy and fibrosis...
2017: PloS One
https://www.readbyqxmd.com/read/28809670/cardio-pulmonary-stethoscope-clinical-validation-with-heart-failure-and-hemodialysis-patients
#13
Magdy F Iskander, Todd B Seto, Ruthsenne Rg Perron, Eunjung Lim, Farhan Qazi
OBJECTIVE: The purpose of this study is to evaluate the accuracy of a noninvasive radiofrequency-based device, the Cardio-Pulmonary Stethoscope (CPS), to monitor heart and respiration rates and detect changes in lung water content in human experiments and clinical trials. METHODS: Three human populations (healthy subjects (n=4), heart failure (n=12) and hemodialysis (n=13) patients) were enrolled in this study. The study was conducted at the University of Hawaii and The Queen's Medical Center in Honolulu, HI...
August 14, 2017: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/28805015/stromelysin-2-mmp-10-a-novel-mediator-of-vascular-remodeling-underlying-pulmonary-hypertension-associated-with-systemic-sclerosis
#14
Jérôme Avouac, Christophe Guignabert, Anna Maria Hoffmann-Vold, Barbara Ruiz, Peter Dorfmuller, Sonia Pezet, Olivia Amar, Ly Tu, Jérôme Van Wassenhove, Jérémy Sadoine, David Launay, Muriel Elhai, Anne Cauvet, Arun Subramaniam, Robert Resnick, Eric Hachulla, Øyvind Molberg, André Kahan, Marc Humbert, Yannick Allanore
OBJECTIVE: To decipher the role of gene candidates involved in pulmonary hypertension (PH) associated with systemic sclerosis (SSc). METHODS: Gene candidates were identified through microarray experiments performed on Affymetrix GeneChip(®) Human Exon 1.0 ST Arrays in endothelial progenitor cell (EPC)-derived endothelial cells (ECs) issued from patients with SSc-PH, SSc without PH and controls. Expression of identified candidates was assessed by quantitative sandwich ELISA in the serum and by immunohistochemistry in lesional lung tissue...
August 13, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28801346/pan-ppar-agonist-iva337-is-effective-in-experimental-lung-fibrosis-and-pulmonary-hypertension
#15
Jerome Avouac, Irena Konstantinova, Christophe Guignabert, Sonia Pezet, Jeremy Sadoine, Thomas Guilbert, Anne Cauvet, Ly Tu, Jean-Michel Luccarini, Jean-Louis Junien, Pierre Broqua, Yannick Allanore
OBJECTIVE: To evaluate the antifibrotic effects of the pan-peroxisome proliferator-activated receptor (PPAR) agonist IVA337 in preclinical mouse models of pulmonary fibrosis and related pulmonary hypertension (PH). METHODS: IVA337 has been evaluated in the mouse model of bleomycin-induced pulmonary fibrosis and in Fra-2 transgenic mice, this latter being characterised by non-specific interstitial pneumonia and severe vascular remodelling of pulmonary arteries leading to PH...
August 11, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28798259/endothelial-cell-related-autophagic-pathways-in-sugen-hypoxia-exposed-pulmonary-arterial-hypertensive-rats
#16
Fumiaki Kato, Seiichiro Sakao, Takao Takeuchi, Toshio Suzuki, Rintaro Nishimura, Tadashi Yasuda, Nobuhiro Tanabe, Koichiro Tatsumi
Pulmonary arterial hypertension (PAH) is characterized by progressive obstructive remodeling of pulmonary arteries. However, no reports have described the causative role of the autophagic pathway in pulmonary vascular endothelial cell (EC) alterations associated with PAH. This study investigated the time-dependent role of the autophagic pathway in pulmonary vascular ECs and pulmonary vascular EC kinesis in a severe PAH rat model (Sugen/Hypoxia rat) and evaluated whether timely induction of the autophagic pathway by rapamycin improves PAH...
August 10, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28795615/international-survey-on-the-perioperative-management-of-pulmonary-endarterectomy-the-perfusion-perspective
#17
Roger D P Stanzel, Johannes Gehron, Matthias Wolff, Natalie Striegl, Peter Roth, Rolf-Hasso Boedeker, Christine Scheibelhut, Johannes Herrmann, Ingeborg Welters, Eckhard Mayer, Matthias Scheffler
INTRODUCTION: Pulmonary endarterectomy (PEA) is the most effective treatment available for chronic thromboembolic pulmonary hypertension (CTEPH). Patient selection, surgical technique and perioperative management have improved patient outcomes, which are traditionally linked to surgical and center experience. However, optimal perfusion care has not been well defined. The goal of the international survey was to better characterize the contemporary perfusion management of PEA and highlight similarities and controversies...
August 1, 2017: Perfusion
https://www.readbyqxmd.com/read/28794135/pulmonary-vasodilator-therapy-is-associated-with-greater-survival-in-eisenmenger-syndrome
#18
Clare Arnott, Geoff Strange, Andrew Bullock, Adrienne C Kirby, Clare O'Donnell, Dorothy J Radford, Leeanne E Grigg, David S Celermajer
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand...
August 9, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28794092/optic-disc-swelling-in-a-patient-with-tuberculous-meningitis-a-diagnostic-challenge
#19
Hanis Zuhaimy, Sue Ngein Leow, Suresh K Vasudevan
We discuss the diagnostic challenge in an adult patient presented with purely ocular symptoms diagnosed with tuberculous meningitis (TBM). A 38-year-old woman presented with bilateral painless blurring of vision. There were bilateral sixth cranial nerve palsy and bilateral optic disc swelling. Optic nerve function tests were normal. Patient was lucid with no signs of meningism. Brain imaging were normal. She had a positive Mantoux test, high erythrocyte sedimentation rate but no clinical evidence of active pulmonary tuberculosis infection...
August 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28793342/risk-of-long-term-infection-related-death-in-clinical-osteoporotic-vertebral-fractures-a-hospital-based-analysis
#20
Ying-Chou Chen, Wei-Che Lin
BACKGROUND: Osteoporotic vertebral fractures adversely impact quality of life and also increase the risk of infection and mortality. Alendronate treatment increases bone mass and reduces the risk of fractures in patients with osteoporosis by suppressing bone resorption. We investigated the relationship between alendronate treatment and infection-related death in patients with osteoporotic vertebral fractures. METHODS: We retrospectively reviewed patients with osteoporosis and vertebral fractures from January 2001 to December 2007...
2017: PloS One
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