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Pulmonary arterial hypertension

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https://www.readbyqxmd.com/read/28091982/death-from-pulmonary-embolism-of-cyanoacrylate-glue-following-gastric-varix-endoscopic-injection
#1
Michael Philip Burke, Chris O'Donnell, Yeliena Baber
We present the case of a 25 year old woman with a complex past medical history including Crigler-Najjar syndrome (Type 1) with a liver transplant in 1993 and subsequent development of cirrhosis with portal hypertension in the transplanted liver. The deceased presented to hospital with hematemesis and investigations showed a large gastric varix. The varix was injected with cyanoacrylate glue. Within 30 min of injection the patient became acutely hypoxic. Urgent chest X-ray demonstrated radio opaque glue within the pulmonary arteries...
January 14, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28090308/treatment-differences-in-pulmonary-arterial-hypertension-management
#2
Bradley A Maron, John J Ryan
No abstract text is available yet for this article.
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090307/hemodynamic-response-to-treatment-of-iron-deficiency-anemia-in-pulmonary-arterial-hypertension-longitudinal-insights-from-an-implantable-hemodynamic-monitor
#3
Muddassir Mehmood, Richa Agarwal, Amresh Raina, Priscilla Correa-Jaque, Raymond L Benza
Despite new therapeutic options, pulmonary arterial hypertension (PAH) remains a progressive disease associated with substantial morbidity and mortality. As such, additional strategies for monitoring and adjunctive management of this disease are important. A 59-year-old woman with scleroderma-associated PAH received an implantable hemodynamic monitor (IHM) as part of a research protocol at our institution. Pulmonary artery pressures, heart rate, and cardiac output (sensor-based algorithm) were measured on a daily basis, and parameters of right ventricular (RV) performance and afterload were calculated...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#4
Julius Koifman, John Granton, John Thenganatt
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090305/resolution-of-myelofibrosis-associated-pulmonary-arterial-hypertension-following-allogeneic-hematopoietic-stem-cell-transplantation
#5
Saadia A Faiz, Cezar Iliescu, Juan Lopez-Mattei, Bela Patel, Lara Bashoura, Uday Popat
We present the case of a 62-year-old man with myelofibrosis-associated pulmonary arterial hypertension (PAH) who underwent allogeneic hematopoietic stem cell transplantation with subsequent resolution of disease and PAH. Right heart catheterization was used to guide PAH therapy before and after transplantation. Drug interactions, adverse effects, and renal insufficiency posed clinical challenges for the management of PAH-specific medications after transplantation. PAH improved soon after transplantation, and vasoactive medications were tapered off...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090304/the-prognostic-significance-of-pulmonary-arterial-capacitance-in-pulmonary-arterial-hypertension-single-center-experience
#6
Nadine Al-Naamani, Ioana R Preston, Nicholas S Hill, Kari E Roberts
This study explores the prognostic utility of pulmonary arterial capacitance (PAC) in a diverse cohort of patients with pulmonary arterial hypertension (PAH) from a tertiary referral center and compares it with the prognostic utility of other hemodynamic parameters. PAC is a strong independent predictor of mortality in patients with PAH.
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090303/genotype-phenotype-effects-of-bmpr2-mutations-on-disease-severity-in-mouse-models-of-pulmonary-hypertension
#7
Andrea L Frump, Arunima Datta, Sampa Ghose, James West, Mark P de Caestecker
More than 350 mutations in the type-2 BMP (bone morphogenetic protein) receptor, BMPR2, have been identified in patients with heritable pulmonary arterial hypertension (HPAH). However, only 30% of BMPR2 mutation carriers develop PAH, and we cannot predict which of these carriers will develop clinical disease. One possibility is that the nature of the BMPR2 mutation affects disease severity. This hypothesis has been difficult to test clinically, given the rarity of HPAH and the complexity of the confounding genetic and environmental risk factors...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090302/three-dimensional-micro-computed-tomography-analysis-of-the-lung-vasculature-and-differential-adipose-proteomics-in-the-sugen-hypoxia-rat-model-of-pulmonary-arterial-hypertension
#8
Kelly J Shields, Kostas Verdelis, Michael J Passineau, Erin M Faight, Lee Zourelias, Changgong Wu, Rong Chong, Raymond L Benza
Pulmonary arterial hypertension (PAH) is a rare disease characterized by significant vascular remodeling. The obesity epidemic has produced great interest in the relationship between small visceral adipose tissue depots producing localized inflammatory conditions, which may link metabolism, innate immunity, and vascular remodeling. This study used novel micro computed tomography (microCT) three-dimensional modeling to investigate the degree of remodeling of the lung vasculature and differential proteomics to determine small visceral adipose dysfunction in rats with severe PAH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090301/pulmonary-pulse-wave-transit-time-is-associated-with-right-ventricular-pulmonary-artery-coupling-in-pulmonary-arterial-hypertension
#9
Kurt W Prins, E Kenneth Weir, Stephen L Archer, Jeremy Markowitz, Lauren Rose, Marc Pritzker, Richard Madlon-Kay, Thenappan Thenappan
Pulmonary pulse wave transit time (pPTT), defined as the time for the systolic pressure pulse wave to travel from the pulmonary valve to the pulmonary veins, has been reported to be reduced in pulmonary arterial hypertension (PAH); however, the underlying mechanism of reduced pPTT is unknown. Here, we investigate the hypothesis that abbreviated pPTT in PAH results from impaired right ventricular-pulmonary artery (RV-PA) coupling. We quantified pPTT using pulsed-wave Doppler ultrasound from 10 healthy age- and sex-matched controls and 36 patients with PAH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090300/bk-channels-in-rat-and-human-pulmonary-smooth-muscle-cells-are-bk%C3%AE-%C3%AE-1-functional-complexes-lacking-the-oxygen-sensitive-stress-axis-regulated-exon-insert
#10
Neil D Detweiler, Li Song, Samantha J McClenahan, Rachel J Versluis, Sujay V Kharade, Richard C Kurten, Sung W Rhee, Nancy J Rusch
A loss of K(+) efflux in pulmonary arterial smooth muscle cells (PASMCs) contributes to abnormal vasoconstriction and PASMC proliferation during pulmonary hypertension (PH). Activation of high-conductance Ca(2+)-activated (BK) channels represents a therapeutic strategy to restore K(+) efflux to the affected PASMCs. However, the properties of BK channels in PASMCs-including sensitivity to BK channel openers (BKCOs)-are poorly defined. The goal of this study was to compare the properties of BK channels between PASMCs of normoxic (N) and chronic hypoxic (CH) rats and then explore key findings in human PASMCs...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090299/tadalafil-therapy-for-sarcoidosis-associated-pulmonary-hypertension
#11
H J Ford, R P Baughman, R Aris, P Engel, J F Donohue
Sarcoidosis-associated pulmonary hypertension (SAPH) is estimated to occur in at least 5% or more of sarcoidosis patients, and it contributes to significant morbidity and mortality. Optimal therapy for SAPH is not well established. We performed a 24-week open-label trial of tadalafil for SAPH at 2 academic medical centers. Subjects were required to have confirmed sarcoidosis plus a right heart catheterization within 12 months of enrollment showing a mean pulmonary artery pressure ≥ 25 mmHg, a pulmonary artery wedge pressure ≤ 15 mmHg, and a calculated pulmonary vascular resistance ≥ 3 Wood units...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090298/in-situ-expression-of-bcl-2-in-pulmonary-artery-endothelial-cells-associates-with-pulmonary-arterial-hypertension-relative-to-heart-failure-with-preserved-ejection-fraction
#12
Raymond L Benza, Gretchen Williams, Changgong Wu, Kelly J Shields, Amresh Raina, Srinivas Murali, Michael J Passineau
We have previously reported that pulmonary artery endothelial cells (PAECs) can be harvested from the tips of discarded Swan-Ganz catheters after right heart catheterization (RHC). In this study, we tested the hypothesis that the existence of an antiapoptotic phenotype in PAECs obtained during RHC is a distinctive feature of pulmonary arterial hypertension (PAH; World Health Organization group 1) and might be used to differentiate PAH from other etiologies of pulmonary hypertension. Specifically, we developed a flow cytometry-based measure of Bcl-2 activity, referred to as the normalized endothelial Bcl-2 index (NEBI)...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090297/exhaled-nitric-oxide-in-pulmonary-arterial-hypertension-associated-with-systemic-sclerosis
#13
Zeling Cao, Stephen C Mathai, Laura K Hummers, Ami A Shah, Fredrick M Wigley, Noah Lechtzin, Paul M Hassoun, Reda E Girgis
The fractional exhaled concentration of nitric oxide (FENO) has been shown to be reduced in idiopathic pulmonary arterial hypertension (PAH) but has not been adequately studied in PAH associated with systemic sclerosis (SSc). We measured FENO at an expiratory flow rate of 50 mL/s in 21 treatment-naive patients with SSc-associated PAH (SSc-PAH), 94 subjects with SSc without pulmonary involvement, and 84 healthy volunteers. Measurements of FENO at additional flow rates of 100, 150, and 250 mL/s were obtained to derive the flow-independent nitric oxide exchange parameters of maximal airway flux (J'awNO) and steady-state alveolar concentration (CANO)...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090295/an-advanced-protocol-driven-transition-from-parenteral-prostanoids-to-inhaled-trepostinil-in-pulmonary-arterial-hypertension
#14
Ronald Oudiz, Manyoo Agarwal, Franz Rischard, Teresa De Marco
Patients with pulmonary arterial hypertension (PAH) often require parenteral prostanoids to improve symptoms and signs of PAH. Complications of parenteral prostanoids-such as catheter-related infections and intolerable adverse effects-may develop, prompting transition to inhaled prostanoids. We report a prospective, protocol-driven transition from parenteral prostanoids to inhaled prostanoids with monitoring of exercise gas exchange and acute hemodynamics. Three PAH centers recruited patients transitioning from parenteral prostanoids to inhaled trepostinil...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090294/the-effects-of-pulmonary-vasodilating-agents-on-right-ventricular-parameters-in-severe-group-3-pulmonary-hypertension-a-pilot-study
#15
Takahiro Sato, Ichizo Tsujino, Ayako Sugimoto, Toshitaka Nakaya, Taku Watanabe, Hiroshi Ohira, Masaru Suzuki, Satoshi Konno, Noriko Oyama-Manabe, Masaharu Nishimura
Pulmonary arterial hypertension (PAH)-approved vasodilators improve right ventricular (RV) function in patients with PAH. However, whether PAH-approved drugs ameliorate RV morphology and function in lung disease-associated pulmonary hypertension (lung-PH) remains unclear. We aimed to prospectively evaluate the changes in RV volume and ejection fraction (RVEF) in 14 consecutive severe lung-PH patients treated with PAH-approved vasodilators. Severe lung-PH was defined as a mean pulmonary arterial pressure (MPAP) of ≥35 mmHg or an MPAP of ≥25 mmHg with a cardiac index (L/min/m(2)) of <2...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090293/relevance-of-angiopoietin-2-and-soluble-p-selectin-levels-in-patients-with-pulmonary-arterial-hypertension-receiving-combination-therapy-with-oral-treprostinil-a-freedom-c2-biomarker-substudy
#16
Manuel J Richter, Ralph Schermuly, Werner Seeger, Youlan Rao, Hossein A Ghofrani, Henning Gall
: Studies have suggested roles for angiopoietin-2 (Ang-2) and soluble P-selectin (sP-selectin) as biomarkers of disease severity and treatment response in pulmonary arterial hypertension (PAH), but additional data are required for validation. We evaluated these biomarkers using data from FREEDOM-C2, in which patients with PAH receiving stable monotherapy or combination therapy were randomized to receive additional treatment with oral treprostinil (up-titrated from 0.25 mg twice daily) or placebo for 16 weeks...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090291/macrophage-migration-inhibitory-factor-as-a-novel-biomarker-of-portopulmonary-hypertension
#17
Hilary M DuBrock, Josanna M Rodriguez-Lopez, Barbara L LeVarge, Michael P Curry, Paul A VanderLaan, Zsuzsanna K Zsengeller, Elizabeth Pernicone, Ioana R Preston, Paul B Yu, Ivana Nikolic, Dihua Xu, Ravi I Thadhani, Richard N Channick, S Ananth Karumanchi
Portopulmonary hypertension (POPH) is a poorly understood complication of liver disease associated with significant morbidity and mortality. We sought to identify novel biomarkers of POPH disease presence and severity. We performed a prospective, multicenter, case-control study involving patients with liver disease undergoing right heart catheterization. POPH cases were defined as a mean pulmonary arterial pressure (mPAP) ≥25 mmHg and pulmonary vascular resistance (PVR) >240 dynes˙s˙cm(-5). Plasma samples were collected from the systemic and pulmonary circulation, and antibody microarray was used to identify biomarkers...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090290/shared-gene-expression-patterns-in-mesenchymal-progenitors-derived-from-lung-and-epidermis-in-pulmonary-arterial-hypertension-identifying-key-pathways-in-pulmonary-vascular-disease
#18
Christa Gaskill, Shennea Marriott, Sidd Pratap, Swapna Menon, Lora K Hedges, Joshua P Fessel, Jonathan A Kropski, DeWayne Ames, Lisa Wheeler, James E Loyd, Anna R Hemnes, Dennis R Roop, Dwight J Klemm, Eric D Austin, Susan M Majka
Rapid access to lung-derived cells from stable subjects is a major challenge in the pulmonary hypertension field, given the relative contraindication of lung biopsy. In these studies, we sought to demonstrate the importance of evaluating a cell type that actively participates in disease processes, as well as the potential to translate these findings to vascular beds in other nonlung tissues, in this instance perivascular skin mesenchymal cells (MCs). We utilized posttransplant or autopsy lung explant-derived cells (ABCG2-expressing mesenchymal progenitor cells [MPCs], fibroblasts) and skin-derived MCs to test the hypothesis that perivascular ABCG2 MPCs derived from pulmonary arterial hypertension (PAH) patient lung and skin would express a gene profile reflective of ongoing vascular dysfunction...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090286/novel-methods-in-pulmonary-hypertension-phenotyping-in-the-age-of-precision-medicine-2015-grover-conference-series
#19
REVIEW
Jarrod W Barnes, Adriano R Tonelli, Gustavo A Heresi, Jennie E Newman, Noël E Mellor, David E Grove, Raed A Dweik
Among pulmonary vascular diseases, pulmonary hypertension (PH) is the best studied and has been the focus of our work. The current classification of PH is based on a relatively simple combination of patient characteristics and hemodynamics. This leads to inherent limitations, including the inability to customize treatment and the lack of clarity from a more granular identification based on individual patient phenotypes. Accurate phenotyping of PH can be used in the clinic to select therapies and determine prognosis and in research to increase the homogeneity of study cohorts...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090285/pulmonary-vascular-and-ventricular-dysfunction-in-the-susceptible-patient-2015-grover-conference-series
#20
REVIEW
Bradley A Maron, Roberto F Machado, Larissa Shimoda
Pulmonary blood vessel structure and tone are maintained by a complex interplay between endogenous vasoactive factors and oxygen-sensing intermediaries. Under physiological conditions, these signaling networks function as an adaptive interface between the pulmonary circulation and environmental or acquired perturbations to preserve oxygenation and maintain systemic delivery of oxygen-rich hemoglobin. Chronic exposure to hypoxia, however, triggers a range of pathogenetic mechanisms that include hypoxia-inducible factor 1α (HIF-1α)-dependent upregulation of the vasoconstrictor peptide endothelin 1 in pulmonary endothelial cells...
December 2016: Pulmonary Circulation
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