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Pulmonary Circulation

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https://www.readbyqxmd.com/read/28632001/vasodilator-responsiveness-in-idiopathic-pulmonary-arterial-hypertension-identifying-a-distinct-phenotype-with-distinct-physiology-and-distinct-prognosis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
(no author information available yet)
No abstract text is available yet for this article.
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597767/increased-mortality-from-complications-of-pulmonary-hypertension-in-patients-undergoing-transcatheter-aortic-valve-replacement
#17
Raj Parikh, Benson Varghese, Huma N Khatoon, Julie A Kovach, Clifford J Kavinsky, Rajive Tandon
Aortic stenosis (AS) leads to pulmonary hypertension (PH) and right ventricle (RV) failure. Our goal was to describe mortality related to postoperative complications in PH patients undergoing transcatheter aortic valve replacement (TAVR). Ninety-three TAVR patients were analyzed (controls, sPAP < 50 mmHg; cases, sPAP ≥ 50 mmHg). Significant findings in cases included increased mortality (365 days), post-TAVR atrioventricular block (AVB) and acute kidney injury (AKI), and increased mean length of stay (LOS)...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597766/identifying-super-responders-in-pulmonary-arterial-hypertension
#18
Stephen J Halliday, Anna R Hemnes
Pharmacotherapeutic options for pulmonary arterial hypertension (PAH) have increased dramatically in the last two decades and along with this have been substantial improvements in survival. Despite these advances, however, PAH remains a progressive and ultimately fatal disease for most patients and only epoprostenol has been shown to improve survival in a randomized control trial. Clinical observations of the heterogeneity of treatment response to different classes of medications across the phenotypically diverse PAH population has led to the identification of patients who derive significantly more benefit from certain medications than the population mean, the so-called "super responders...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597765/poor-survival-in-patients-with-scleroderma-and-pulmonary-hypertension-due-to-heart-failure-with-preserved-ejection-fraction
#19
Khalil I Bourji, Benjamin W Kelemen, Stephen C Mathai, Rachel L Damico, Todd M Kolb, Valentina Mercurio, Franco Cozzi, Ryan J Tedford, Paul M Hassoun
Pulmonary hypertension due to heart failure with preserved ejection fraction (PH-HFpEF) has been poorly studied in patients with systemic sclerosis (SSc). We sought to compare clinical characteristics and survival of SSc patients with PH-HFpEF (SSc-PH-HFpEF) versus pulmonary arterial hypertension (SSc-PAH). We hypothesized that patients with SSc-PH-HFpEF have a similar poor overall prognosis compared with patients with SSc-PAH when matched for total right ventricular load. The analysis included 117 patients with SSc-PH (93 with SSc-PAH versus 24 with SSc-PH-HFpEF) enrolled prospectively in the Johns Hopkins PH Registry...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597764/microct-analysis-of-vascular-morphometry-a-comparison-of-right-lung-lobes-in-the-sugen-hypoxic-rat-model-of-pulmonary-arterial-hypertension
#20
Erin M Faight, Kostas Verdelis, Lee Zourelias, Rong Chong, Raymond L Benza, Kelly J Shields
Pulmonary arterial hypertension (PAH) is a rare disease characterized by significant vascular remodeling within the lung. Clinical computed tomography (CT) scans are routinely used to aid in PAH diagnosis. Animal models, including the Sugen-hypoxic rat model (SU/hyp), of PAH closely mimic human PAH development. We have previously used micro-computed tomography (microCT) to find extensive right lung vascular remodeling in the SU/hyp. We hypothesized that the individual right lung lobes may not contribute equally to overall lung vascular remodeling...
April 2017: Pulmonary Circulation
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