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Pulmonary Circulation

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https://www.readbyqxmd.com/read/28704134/pulmonary-vascular-effect-of-insulin-in-a-rodent-model-of-pulmonary-arterial-hypertension
#1
Aaron W Trammell, Megha Talati, Thomas R Blackwell, Niki L Fortune, Kevin D Niswender, Joshua P Fessel, John H Newman, James D West, Anna R Hemnes
Pulmonary arterial hypertension (PAH) is associated with metabolic derangements including insulin resistance, although their effects on the cardiopulmonary disease are unclear. We hypothesized that insulin resistance promotes pulmonary hypertension (PH) development and mutations in type 2 bone morphogenetic protein receptor (BMPR2) cause cellular insulin resistance. Using a BMPR2 transgenic murine model of PAH and two models of inducible diabetes mellitus, we explored the impact of hyperglycemia and/or hyperinsulinemia on development and severity of PH...
August 7, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28660794/left-main-coronary-artery-compression-by-a-dilated-main-pulmonary-artery-and-left-coronary-sinus-of-valsalva-aneurysm-in-a-patient-with-heritable-pulmonary-arterial-hypertension-and-flna-mutation
#2
Akihiro Hirashiki, Shiro Adachi, Yoshihisa Nakano, Yoshihiro Kamimura, Takeshi Ogo, Norifumi Nakanishi, Takayuki Morisaki, Hiroko Morisaki, Atsuya Shimizu, Kenji Toba, Toyoaki Murohara, Takahisa Kondo
Left main coronary artery (LMCA) disease due to external compression by a dilated main pulmonary artery (MPA) is an uncommon clinical entity. Here, we describe a 52-year-old woman with pulmonary arterial hypertension (PAH) and anteroseptal old myocardial infarction (OMI). The cause of the OMI was external compression of the LMCA by the dilated MPA and aneurysm of the left coronary sinus of Valsalva. The patient's sister (aged 56 years) had also been diagnosed with PAH and both women had a novel heterozygous splicing mutation, IVS2-2A > G (c...
June 29, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28660793/pulmonary-hypertension-in-patients-with-chronic-kidney-disease-invasive-hemodynamic-etiology-and-outcomes
#3
Jared M O'Leary, Tufik R Assad, Meng Xu, Kelly A Birdwell, Eric Farber-Eger, Quinn S Wells, Anna R Hemnes, Evan L Brittain
Pulmonary hypertension (PH) is common in patients with chronic kidney disease (CKD) and associated with increased mortality but the hemodynamic profiles, clinical risk factors, and outcomes have not been well characterized. Our objective was to define the hemodynamic profile and related risk factors for PH in CKD patients. We extracted clinical and hemodynamic data from Vanderbilt's de-identified electronic medical record on all patients undergoing right heart catheterization during 1998-2014. CKD (stages III-V) was defined by estimated glomerular filtration rate thresholds...
June 29, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28644070/particulate-matter-disrupts-human-lung-endothelial-cell-barrier-integrity-via-rho-dependent-pathways
#4
Ting Wang, Yuka Shimizu, Xiaomin Wu, Gabriel T Kelly, Xiaoyan Xu, Lichun Wang, Zhongqing Qian, Yin Chen, Joe G N Garcia
Increased exposure to ambient particulate matter (PM) is associated with elevated morbidity and mortality in patients with cardiopulmonary diseases and cancer. We and others have shown that PM induces lung microvascular barrier dysfunction which potentially enhances the systemic toxicity of PM. However, the mechanisms by which PM disrupts vascular endothelial integrity remain incompletely explored. We hypothesize that PM induces endothelial cell (EC) cytoskeleton rearrangement via Rho GTPase-dependent pathways to facilitate vascular hyperpermeability...
June 23, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28632001/vasodilator-responsiveness-in-idiopathic-pulmonary-arterial-hypertension-identifying-a-distinct-phenotype-with-distinct-physiology-and-distinct-prognosis
#5
David Langleben, Stylianos Orfanos
Within the cohort of patients suffering from idiopathic pulmonary arterial hypertension (IPAH) is a group that responds dramatically (VR-PAH) to an acute vasodilator challenge and that has excellent long-term hemodynamic improvement and prognosis on high dose calcium channel blockers compared with vasodilator non-responders (VN-PAH). For the purposes of diagnosing VR-PAH, there is to date no test to replace the acute vasodilator challenge. However, recent studies have identified markers that may aid in the identification of VR-PAH, including peripheral blood lymphocyte RNA expression levels of desmogelin-2 and Ras homolog gene family member Q, and plasma levels of provirus integration site for Moloney murine leukemia virus...
June 20, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28628000/epigenetics-inflammation-and-metabolism-in-right-heart-failure-associated-with-pulmonary-hypertension
#6
Nolwenn Samson, Roxane Paulin
Right ventricular failure (RVF) is the most important prognostic factor for both morbidity and mortality in pulmonary arterial hypertension (PAH), but also occurs in numerous other common diseases and conditions, including left ventricle dysfunction. RVF remains understudied compared with left ventricular failure (LVF). However, right and left ventricles have many differences at the morphological level or the embryologic origin, and respond differently to pressure overload. Therefore, knowledge from the left ventricle cannot be extrapolated to the right ventricle...
June 19, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28621582/assessment-of-pulmonary-arterial-compliance-evaluated-using-harmonic-oscillator-kinematics
#7
Yasunobu Hayabuchi, Akemi Ono, Yukako Homma, Shoji Kagami
We hypothesized that KPA, a harmonic oscillator kinematics-derived spring constant parameter of the pulmonary artery pressure (PAP) profile, reflects PA compliance in pediatric patients. In this prospective study of 33 children (age range = 0.5-20 years) with various cardiac diseases, we assessed the novel parameter designated as KPA calculated using the pressure phase plane and the equation KPA = (dP/dt_max)(2)/([Pmax - Pmin])/2)(2), where dP/dt_max is the peak derivative of PAP, and Pmax - Pmin is the difference between the minimum and maximum PAP...
June 16, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597780/novel-approach-to-classifying-patients-with-pulmonary-arterial-hypertension-using-cluster-analysis
#8
Kishan S Parikh, Youlan Rao, Tariq Ahmad, Kai Shen, G Michael Felker, Sudarshan Rajagopal
Pulmonary arterial hypertension (PAH) patients have distinct disease courses and responses to treatment, but current diagnostic and treatment schemes provide limited insight. We aimed to see if cluster analysis could distinguish clinical phenotypes in PAH. An unbiased cluster analysis was performed on 17 baseline clinical variables of PAH patients from the FREEDOM-M, FREEDOM-C, and FREEDOM-C2 randomized trials of oral treprostinil versus placebo. Participants were either treatment-naïve (FREEDOM-M) or on background therapy (FREEDOM-C, FREEDOM-C2)...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597779/phosphodiesterase-type-5-inhibitor-to-riociguat-transition-is-associated-with-hemodynamic-and-symptomatic-improvement-in-pulmonary-hypertension
#9
Ryan Davey, Raymond L Benza, Srinivas Murali, Amresh Raina
Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. We studied the clinical and hemodynamics effects of transitioning 12 pulmonary hypertension patients from Phosphodiesterase type 5 inhibitor (PDE5i) to riociguat, and demonstrated a significant increase in cardiac index, fall in pulmonary vascular resistance, and improvement in functional class with this switch. Switch from PDE5i to riociguat appeared to be safe and fairly well tolerated in most patients...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597778/genomic-stability-of-pulmonary-artery-endothelial-colony-forming-cells-in-culture
#10
Kylie M Drake, Chiara Federici, Heng T Duong, Suzy A Comhair, Serpil C Erzurum, Kewal Asosingh, Micheala A Aldred
Pulmonary vascular remodeling, including proliferation and migration of pulmonary artery endothelial cells (PAEC), is a pathologic hallmark of pulmonary arterial hypertension (PAH). Multiple studies have shown evidence of increased levels of DNA damage and lineage-specific genetic changes in PAH lung vascular cells, suggesting increased genomic instability. Highly proliferative endothelial colony-forming cell (ECFC) clones can be isolated from PAEC. Here we utilized ECFC to track chromosomal copy number of 20 PAH and eight control clones across serial passages using genome-wide microarrays...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597777/improved-metabolism-and-redox-state-with-a-novel-preservation-solution-implications-for-donor-lungs-after-cardiac-death-dcd
#11
David A Schipper, Anthony V Louis, Destiny S Dicken, Kitsie Johnson, Ryszard T Smolenski, Stephen M Black, Ray Runyan, John Konhilas, Joe G N Garcia, Zain Khalpey
Lungs donated after cardiac death (DCD) are an underutilized resource for a dwindling donor lung transplant pool. Our study investigates the potential of a novel preservation solution, Somah, to better preserve statically stored DCD lungs, for an extended time period, when compared to low-potassium dextran solution (LPD). We hypothesize that Somah is a metabolically superior organ preservation solution for hypothermic statically stored porcine DCD lungs, possibly improving lung transplant outcomes. Porcine DCD lungs (n = 3 per group) were flushed with and submerged in cold preservation solution...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597776/the-pulmonary-vascular-research-institute-celebrates-its-first-decade
#12
Jason X-J Yuan, Nicholas W Morrell, Kurt R Stenmark, Irene M Lang, Ghazwan Butrous, Mary E Reynolds
No abstract text is available yet for this article.
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597775/dobutamine-stress-mri-in-pulmonary-hypertension-relationships-between-stress-pulmonary-artery-relative-area-change-rv-performance-and-10-year-survival
#13
Kevin G Blyth, Alessandro Bellofiore, Geeshath Jayasekera, John E Foster, Tracey Steedman, Naomi C Chesler, Andrew J Peacock
In pulmonary hypertension (PH), right ventricular (RV) performance determines survival. Pulmonary artery (PA) stiffening is an important biomechanical event in PH and also predicts survival based on the PA relative area change (RAC) measured at rest using magnetic resonance imaging (MRI). In this exploratory study, we sought to generate novel hypotheses regarding the influence of stress RAC on PH prognosis and the interaction between PA stiffening, RV performance and survival. Fifteen PH patients underwent dobutamine stress-MRI (ds-MRI) and right heart catheterization...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597774/combination-therapy-in-pulmonary-arterial-hypertension-recent-accomplishments-and-future-challenges
#14
Annie-Christine Lajoie, Sebastien Bonnet, Steeve Provencher
Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by a progressive increase in pulmonary vascular resistance, ultimately leading to right heart failure and death. Throughout the past 20 years, numerous specific pharmacologic agents, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostaglandins, and more recently, soluble guanylate cyclase stimulators and selective IP prostacyclin receptor agonist, have emerged for the treatment of PAH. Early clinical trials were typically of short-term duration, comparing the effects of PAH-targeted therapies versus placebo and using exercise tolerance as the primary endpoint in most trials...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597773/prostacyclins-have-no-direct-inotropic-effect-on-isolated-atrial-strips-from-the-normal-and-pressure-overloaded-human-right-heart
#15
Sarah Holmboe, Asger Andersen, Rebekka V Jensen, Hans Henrik Kimose, Lars B Ilkjær, Lei Shen, Lucie H Clapp, Jens Erik Nielsen-Kudsk
Prostacyclins are vasodilatory agents used in the treatment of pulmonary arterial hypertension. The direct effects of prostacyclins on right heart function are still not clarified. The aim of this study was to investigate the possible direct inotropic properties of clinical available prostacyclin mimetics in the normal and the pressure-overloaded human right atrium. Trabeculae from the right atrium were collected during surgery from chronic thromboembolic pulmonary hypertension (CTEPH) patients with pressure-overloaded right hearts, undergoing pulmonary thromboendarterectomy (n = 10) and from patients with normal right hearts operated by valve replacement or coronary bypass surgery (n = 9)...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597772/right-ventricular-st-elevation-myocardial-infarction-as-a-cause-of-death-in-idiopathic-pulmonary-arterial-hypertension
#16
Yang Zhan, Barry Burstein, Ali O Abualsaud, Mohamed Nosair, Andrew M Hirsch, Lyda Lesenko, David Langleben
A 32-year-old woman with advanced idiopathic pulmonary arterial hypertension (PAH), treated with oral tadalafil and intravenous epoprostenol, presented with typical angina pectoris of one day's duration. Her electrocardiogram, previously typical of pulmonary hypertension, revealed an acute ST-elevation myocardial infarction in the anterior precordial leads. She had a prior coronary angiogram, in preparation for lung transplantation, that revealed normal coronary arteries. Urgent coronary angiography showed acute occlusion of several acute marginal coronary branches that feed the right ventricle (RV)...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597771/first-in-child-use-of-the-oral-selective-prostacyclin-ip-receptor-agonist-selexipag-in-pulmonary-arterial-hypertension
#17
Lianne M Geerdink, Harald Bertram, Georg Hansmann
Pulmonary arterial hypertension (PAH) is a complex disease with a poor prognosis. Selexipag is a selective prostacyclin receptor agonist with vasodilatory, anti-proliferative, anti-inflammatory, and pro-angiogenic properties. However, no clinical data on its therapeutic use in children with PAH are currently available. Here, we report the case of a 12-year-old girl who presented in World Health Organization (WHO) functional class III and right ventricular (RV) failure with recurrent syncope, dizziness, and progressive fatigue for two years...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597770/pulmonary-arterial-hypertension-specialists-knowledge-practices-and-attitudes-of-genetic-counseling-and-genetic-testing-in-the-usa
#18
Joseph E Jacher, Lisa J Martin, Wendy K Chung, James E Loyd, William C Nichols
Pulmonary arterial hypertension (PAH) is characterized by obstruction of pre-capillary pulmonary arteries, which leads to sustained elevation of pulmonary arterial pressure. Identifying those at risk through early interventions, such as genetic testing, may mitigate disease course. Current practice guidelines recommend genetic counseling and offering genetic testing to individuals with heritable PAH, idiopathic PAH, and their family members. However, it is unclear if PAH specialists follow these recommendations...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597769/a-systematic-review-of-transition-studies-of-pulmonary-arterial-hypertension-specific-medications
#19
Avraham Sofer, Michael J Ryan, Ryan J Tedford, Joel A Wirth, Wassim H Fares
Pulmonary arterial hypertension (PAH) is a progressive potentially fatal disease. Multiple pharmacologic options are now available, which facilitated transitions between different therapeutic options, although the evidence for such transitions has not been well described. We sought to review the evidence supporting the safety and/or efficacy of transitioning between PAH-specific medications. We performed a systematic review of all published studies in the Medline database between 1 January 2000 and 30 June 2016 reporting on any transition between the currently Food and Drug Administration (FDA)-approved PAH-specific medications...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597768/corrigendum
#20
(no author information available yet)
No abstract text is available yet for this article.
April 2017: Pulmonary Circulation
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