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"Pulmonary hypertension" OR "Pulmonary arterial hypertension"

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https://www.readbyqxmd.com/read/28087362/clinical-phenotypes-and-outcomes-of-heritable-and-sporadic-pulmonary-veno-occlusive-disease-a-population-based-study
#1
David Montani, Barbara Girerd, Xavier Jaïs, Marilyne Levy, David Amar, Laurent Savale, Peter Dorfmüller, Andrei Seferian, Edmund M Lau, Mélanie Eyries, Jérôme Le Pavec, Florence Parent, Damien Bonnet, Florent Soubrier, Elie Fadel, Olivier Sitbon, Gérald Simonneau, Marc Humbert
BACKGROUND: Bi-allelic mutations of the EIF2AK4 gene cause heritable pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis (PVOD/PCH). We aimed to assess the effect of EIF2AK4 mutations on the clinical phenotypes and outcomes of PVOD/PCH. METHODS: We did a population-based study using clinical, functional, and haemodynamic data from the registry of the French Pulmonary Hypertension Network. We reviewed the clinical data and outcomes from all patients referred to the French Referral Centre (Pulmonary Department, Hospital Kremlin-Bicêtre, University Paris-Sud) with either confirmed or highly probable PVOD/PCH with DNA available for mutation screening (excluding patients with other risk factors of pulmonary hypertension, such as chronic respiratory diseases)...
January 10, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28087179/value-of-serum-n-terminal-b-type-natriuretic-peptide-in-asymptomatic-structural-heart-disease-in-taiwanese-population-comparisons-with-current-esc-guidelines
#2
Ta-Chuan Hung, Kuang-Te Wang, Chun-Ho Yun, Jen-Yuan Kuo, Charles Jia-Yin Hou, Chia-Yuan Liu, Tung-Hsin Wu, Hiram G Bezerra, Hsiao-Yang Cheng, Chung-Lieh Hung, Hung-I Yeh
BACKGROUND: The relationship between N-terminal pro-brain natriuretic peptide (NT-proBNP) and cardiac structural or functional anomalies in pre-clinical, asymptomatic Asian populations has not been well identified. METHODS: From October 2005 to March 2008, we enrolled consecutive asymptomatic adults with preserved global left ventricular (LV) function (ejection fraction>50%) who underwent annual cardiovascular health survey. Circulating NT-proBNP was used to identify echo-defined cardiac structural/functional anomalies and compared to current recommended cut-off from the European Society of Heart Failure...
December 29, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28081353/clinical-correlation-between-the-6-min-walk-test-andcardiopulmonary-exercise-testing-in-patients-with-pulmonary-arterial-hypertension
#3
Serap Acar, Sema Savcı, Didem Kardibak, Buse Özcan Kahraman, Bahri Akdeniz, Ebru Özpelit, Can Sevinç
BACKGROUND/AIM: The aims of the present study were to assess the relationship between the distance walked during the 6-min walk test (6MWT) and exercise capacity as determined by cardiopulmonary exercise testing (CPET) in patients with pulmonary arterial hypertension (PAH) and to investigate the prognostic value of the 6MWT in comparison to clinical parameters of CPET and echocardiography findings. MATERIALS AND METHODS: Thirty PAH patients participated in the study...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28081336/the-factors-affecting-survival-in-patients-with-bronchiectasis
#4
Fatma Çiftçi, Elif Şen, Sevgi Behiye Saryal, Zeynep Pınar Önen, Banu Gülbay, Öznur Yıldız, Turan Acıcan, Gülseren Karabıyıkoğlu
BACKGROUND/AIM: There is limited information about the rate and modifiers of mortality in noncystic fibrosis bronchiectasis. MATERIALS AND METHODS: This study enrolled a total of 56 bronchiectasis patients. Patients' body mass index, smoking habit, previous therapies, comorbid disorders, history of vaccination, bronchiectasis type and radiological extent, arterial blood gas analysis, respiratory function tests, and laboratory results were recorded. RESULTS: After a follow-up of 65...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28079864/bronchopulmonary-dysplasia-and-pulmonary-hypertension-a-meta-analysis
#5
G Al-Ghanem, P Shah, S Thomas, L Banfield, S El Helou, C Fusch, A Mukerji
OBJECTIVE: Pulmonary hypertension (PH) is a complication of bronchopulmonary dysplasia (BPD) but the true impact of PH in patients with BPD remains unclear. We sought to systematically review and meta-analyze incidence of PH in BPD and compare clinical outcomes of BPD patients with PH to those without PH in preterm infants. STUDY DESIGN: Medline, Embase, PsychINFO and CINAHL were searched from January 2000 through December 2015. Cohort, case-control and randomized studies were included...
January 12, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28079843/the-association-between-bnp-6mwd-test-dlco-and-pulmonary-hypertension-in-sarcoidosis
#6
Mehdi Mirsaeidi, Hesham R Omar, Robert Baughman, Roberto Machado, Nadera Sweiss
INTRODUCTION: Pulmonary hypertension (PH) is a feared complication in patients with sarcoidosis.It is an important negative prognostic factor which is reflected as a priority given to these subjects for orthotopic lung transplantation. We evaluated the relationship between BNP, 6MWD test, DLCO% values and the severity of sarcoidosis-associated pulmonary hypertension. METHODS: A retrospective chart review was conducted between January 2010 and January 2015 on consecutive confirmed adult subjects diagnosed with sarcoidosis...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079786/fatal-postoperative-systemic-pulmonary-hypertension-in-benfluorex-induced-valvular-heart-disease-surgery-a-case-report
#7
Christophe Baufreton, Patrick Bruneval, Marie-Christine Rousselet, Pierre-Vladimir Ennezat, Olivier Fouquet, Raphael Giraud, Carlo Banfi
RATIONALE: Drug-induced valvular heart disease (DI-VHD) remains an under-recognized entity. PATIENT CONCERNS: This report describes a heart valve replacement which was complicated by intractable systemic pulmonary arterial hypertension in a 61-year-old female with severe restrictive mitral and aortic disease. The diagnosis of valvular disease was preceded by a history of unexplained respiratory distress. The patient had been exposed to benfluorex for 6.5 years. DIAGNOSES: The diagnostic procedure documented specific drug-induced valvular fibrosis...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28079595/acetylated-cyclophilin-a-is-a-major-mediator-in-hypoxia-induced-autophagy-and-pulmonary-vascular-angiogenesis
#8
Min Mao, Xiufeng Yu, Xin Ge, Rui Gu, Qian Li, Shasha Song, Xiaodong Zheng, Tingting Shen, Xuecang Li, Yao Fu, Jiali Li, Daling Zhu
BACKGROUND: Autophagy is a major intracellular degradation and recycling process that maintains cellular homeostasis, which is involved in structural and functional abnormalities of pulmonary vasculature in hypoxic pulmonary arterial hypertension (HPAH). Cyclophilin A (CyPA) is a secreted, oxidative stress-induced factor. Its role in inducing autophagy and augmenting endothelial cell dysfunction has never been explored. METHODS: Lungs from rats exposed to chronic hypoxia were examined for autophagy with electron microscopy, western blotting, and fluorescence microscopy...
January 12, 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28078552/pediatric-development-of-bosentan-facilitated-by-modeling-and-simulation
#9
Jochen Zisowsky, Martine Géhin, Andjela Kusic-Pajic, Andreas Krause, Maurice Beghetti, Jasper Dingemanse
BACKGROUND: Bosentan is approved for use in adult patients with pulmonary arterial hypertension. The primary aim of the pharmacokinetic modeling was the provision of a systematic guidance for study design and enhanced understanding of pharmacokinetics across the entire pediatric age range. METHODS: A physiologically based pharmacokinetic model was developed for the pediatric population; starting from an adult model, the effects of body weight, age, and maturation of relevant metabolizing enzymes were incorporated to extrapolate the pharmacokinetics to children...
January 11, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28078487/the-role-of-the-nox4-derived-ros-mediated-rhoa-rho-kinase-pathway-in-rat-hypertension-induced-by-chronic-intermittent-hypoxia
#10
Wen Lu, Jing Kang, Ke Hu, Si Tang, Xiufang Zhou, Lifang Xu, Yuanyuan Li, Shuhui Yu
BACKGROUND: Obstructive sleep apnea syndrome, which is a risk factor for resistant hypertension, is characterized by chronic intermittent hypoxia (CIH) and is associated with many cardiovascular diseases. CIH elicits systemic oxidative stress and sympathetic hyperactivity, which lead to hypertension. Rho kinases (ROCKs) are considered to be major effectors of the small GTPase RhoA and have been extensively studied in the cardiovascular field. Upregulation of the RhoA/ROCK signaling cascade is observed in various cardiovascular disorders, such as atherosclerosis, pulmonary hypertension, and stroke...
January 11, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28077532/modern-hydroxyethyl-starch-and-acute-kidney-injury-after-cardiac-surgery-a-prospective-multicentre-cohort
#11
M Vives, R Callejas, P Duque, G Echarri, D N Wijeysundera, A Hernandez, A Sabate, M Bes-Rastrollo, P Monedero
BACKGROUND: Recent trials have shown hydroxyethyl starch (HES) solutions increase the risk of acute kidney injury (AKI) in critically ill patients. It is uncertain whether these adverse effects also affect surgical patients. We sought to determine the renal safety of modern tetrastarch (6% HES 130/0.4) use in cardiac surgical patients. METHODS: In this multicentre prospective cohort study, 1058 consecutive patients who underwent cardiac surgery from 15th September 2012 to 15th December 2012 were recruited in 23 Spanish hospitals...
October 2016: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28077486/severe-pulmonary-arterial-hypertension-in-a-patient-with-small-ventricular-septal-defect
#12
Narayanapillai Jayaprasad, Suresh Madhavan
No abstract text is available yet for this article.
January 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28077433/osteoprotegerin-disruption-attenuates-hysu-induced-pulmonary-hypertension-through-integrin-%C3%AE-v%C3%AE-3-fak-akt-pathway-suppression
#13
Daile Jia, Qian Zhu, Huan Liu, Caojian Zuo, Yuhu He, Guilin Chen, Ankang Lu
BACKGROUND: Pulmonary arterial remodeling characterized by increased vascular smooth muscle proliferation is commonly seen in life-threatening disease, pulmonary arterial hypertension (PAH). Clinical studies have suggested a correlation between osteoprotegerin serum levels and PAH severity. Here, we aimed to invhestigate vascular osteoprotegerin expression and its effects on pulmonary arterial smooth muscle cell proliferation in vitro and in vivo, as well as examine the signal transduction pathways mediating its activity...
February 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28076893/-pseudo-feeders-on-fetal-mri-predict-outcome-in-vein-of-galen-malformations
#14
Guillaume Saliou, Irène Vraka, Jean-Paul Teglas, Marie-Victoire Senat, Philippe Durand, Claire Colmant, Augustin Ozanne, Jelena Martinovic, Pierre Tissiere, Catherine Adamsbaum
OBJECTIVE: While vein of Galen aneurysmal malformations (VGAM) can be diagnosed in the fetus, the challenge is predicting the occurrence of its two major complications: cardiopulmonary failure and encephalomalacia. This study attempts to determine which fetal brain MRI features might be used to predict the development of these complications at birth. METHODS: The cohort was extracted from a prospectively-assembled database of VGAM cases managed at a single referral centre from 2000 to 2014...
January 11, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28074408/pharmacologic-stress-myocardial-perfusion-imaging-in-patients-with-pulmonary-hypertension-what-do-we-know-and-what-remains-to-be-learned
#15
EDITORIAL
Andrew Van Tosh, Kenneth J Nichols
No abstract text is available yet for this article.
January 10, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28073593/integrative-cardiopulmonary-ultrasound-for-interstitial-lung-disease-assessment-correlation-between-lung-ultrasound-performance-and-cardiac-involvement
#16
Wei-Wei Zhu, Yi-Dan Li, Hong Li, Xiu-Zhang Lu, Ling-Yun Kong, Xiao-Guang Ye, Qi-Zhe Cai, Lan-Lan Sun, Wei Jiang, Li Wang
The aims of this study were to apply integrative cardiopulmonary ultrasound (thoracic ultrasound) to the evaluation of patients with interstitial lung disease (ILD) and to determine the relationship between lung ultrasound signs and echocardiographic parameters such as systolic pulmonary artery pressure (SPAP) and various right ventricular parameters. ILD patients and healthy controls underwent lung ultrasound (LUS) and echocardiographic tests. In addition to traditional echocardiographic parameters, right ventricular free wall longitudinal strain (RVLS_FW) was measured using 2-D speckle-tracking echocardiography...
January 7, 2017: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/28073030/to-screen-or-not-to-screen-for-chronic-thromboembolic-pulmonary-hypertension-after-acute-pulmonary-embolism
#17
REVIEW
Yvonne M Ende-Verhaar, Menno V Huisman, Frederikus A Klok
Chronic thromboembolic pulmonary hypertension (CTEPH) is the most severe long term complication of acute pulmonary embolism (PE). Untreated, CTEPH is associated with a very poor prognosis and high risk of mortality, although curation can be achieved by surgical removal of the obstructive endothelialised thromboemboli from the pulmonary arteries. Early CTEPH diagnosis may improve surgical possibilities and patients outcome. Currently, early diagnosis of CTEPH is a major challenge as demonstrated by an unacceptable median diagnostic delay of over a year and as a result, surgery is impossible in 40% of patients...
January 5, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28072955/-clinical-analysis-of-12-patients-with-pediatric-antiphospholipid-syndrome-with-pulmonary-embolism
#18
J R Ma, H M Song, J Xiao, X Y Tang, Y Y He, M Wei
Objective: To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism. Method: Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism. Result: Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28072745/safety-and-efficacy-of-chloral-hydrate-for-conscious-sedation-of-infants-in-the-pediatric-cardiovascular-intensive-care-unit
#19
Mei-Lian Chen, Qiang Chen, Fan Xu, Jia-Xin Zhang, Xiao-Ying Su, Xiao-Zhen Tu
This study evaluates the safety and efficacy of chloral hydrate administration for the conscious sedation of infants in the pediatric cardiovascular intensive care unit (PCICU).We conducted a retrospective review of the charts of 165 infants with congenital heart disease who received chloral hydrate in our PCICU between January 2014 and December 2014. Chloral hydrate was administered orally or rectally to infants using doses of 50 mg/kg. We collected and analyzed relevant clinical parameters.The overall length of time to achieve sedation was ranged from 5 to 35 min (10...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28072531/discovery-of-2-1r-4r-4-4-chlorophenyl-phenyl-carbamoyl-oxy-methyl-cyclohexyl-methoxy-acetate-ralinepag-an-orally-active-prostacyclin-receptor-agonist-for-the-treatment-of-pulmonary-arterial-hypertension
#20
Thuy-Anh Tran, Bryan Kramer, Young-Jun Shin, Pureza Vallar, P Douglas Boatman, Ning Zou, Carleton R Sage, Tawfik Gharbaoui, Ashwin Krishnan, Biman Pal, Sagar R Shakya, Antonio Garrido Montalban, John Wesley Adams, Juan Ramirez, Dominic Peter Behan, Anna Shifrina, Anthony Blackburn, Tina Leakakos, Yunqing Shi, Michael Morgan, Abu Sadeque, Weichao Chen, David John Unett, Ibragim Gaidarov, Xiaohua Chen, Steve Chang, Hsin-Hui Shu, Shiu-Feng Tung, Graeme Semple
The design and synthesis of a new series of potent non-prostanoid IP receptor agonists that showed oral efficacy in the rat monocrotaline model of pulmonary arterial hypertension (PAH) is described. Detailed profiling of a number of analogues resulted in the identification of 3c (APD811, Ralinepag) that has good selectivity in both binding and functional assays with respect to most members of the prostanoid receptor family, and a more modest 40- 50-fold selectivity over the EP3 receptor. In our hands, its potency and efficacy are superior in vitro to MRE269 (the active metabolite of the clinical compound NS-304) in multiple in vitro measures of IP receptor mediated cAMP signaling...
January 10, 2017: Journal of Medicinal Chemistry
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