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Pulmonary Circulation

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October 17, 2017: Pulmonary Circulation
Josephine Pickworth, Alexander Rothman, James Iremonger, Helen Casbolt, Kay Hopkinson, Peter M Hickey, Santhi Gladson, Sheila Shay, Nicholas W Morrell, Sheila E Francis, James D West, Allan Lawrie
Bone morphogenetic protein receptor type 2 (BMPR2) mutations are present in patients with heritable and idiopathic pulmonary arterial hypertension (PAH). Circulating levels of interleukin-1 (IL-1) are raised in patients and animal models. Whether interplay between BMP and IL-1 signaling can explain the local manifestation of PAH in the lung remains unclear. Cell culture, siRNA, and mRNA microarray analysis of RNA isolated from human pulmonary artery (PASMC) and aortic (AoSMC) smooth muscle cells were used. R899X(+/-) BMPR2 transgenic mice fed a Western diet for six weeks were given daily injections of IL-1ß prior to assessment for PAH and tissue collection...
September 22, 2017: Pulmonary Circulation
Nadine Al-Naamani, Jessica K Paulus, Kari E Roberts, Michael W Pauciulo, Katie Lutz, William C Nichols, Steven M Kawut
This study explores the racial and ethnic differences in presentation, severity, and treatment of patients with pulmonary arterial hypertension (PAH) in a large multicenter registry. African American and Hispanic patients are more likely to present with associated PAH compared to non-Hispanic whites. Hispanic patients with PAH were less likely to be treated with PAH-specific medications compared to non-Hispanic whites.
September 15, 2017: Pulmonary Circulation
Rudolf K F Oliveira, Mariana Faria-Urbina, Bradley A Maron, Mario Santos, Aaron B Waxman, David M Systrom
Borderline resting mean pulmonary arterial pressure (mPAP) is associated with adverse outcomes and affects the exercise pulmonary vascular response. However, the pathophysiological mechanisms underlying exertional intolerance in borderline mPAP remain incompletely characterized. In the current study, we sought to evaluate the prevalence and functional impact of exercise pulmonary hypertension (ePH) across a spectrum of resting mPAP's in consecutive patients with contemporary resting right heart catheterization (RHC) and invasive cardiopulmonary exercise testing...
July 2017: Pulmonary Circulation
Haiyang Tang, Rebecca R Vanderpool, Jian Wang, Jason X-J Yuan
No abstract text is available yet for this article.
July 2017: Pulmonary Circulation
Thenappan Thenappan
No abstract text is available yet for this article.
July 2017: Pulmonary Circulation
Horst Olschewski
No abstract text is available yet for this article.
July 2017: Pulmonary Circulation
Rebecca Johnson Kameny, Elizabeth Colglazier, Hythem Nawaytou, Phillip Moore, V Mohan Reddy, David Teitel, Jeffrey R Fineman
Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease as a consequence of altered pulmonary hemodynamics with increased pulmonary blood flow and pressure. The development of pulmonary vascular disease (PVD) in this patient population is an important concern in determining operative strategy. Early, definitive surgical repair, when possible, is the best therapy to prevent and treat PVD. However, this is not possible in some patients because they either presented late, after the development of PVD, or they have complex lesions not amenable to one-step surgical correction, including patients with single ventricle physiology, who have a continuing risk of developing PVD...
July 2017: Pulmonary Circulation
Anna D T Muntingh, Sylvia Gerritsen, Neeltje M Batelaan, Harm Jan Bogaard
Pulmonary arterial hypertension (PAH) affects the physical and emotional wellbeing of patients. More information is needed regarding mental problems and preferences for support. Qualitative interviews were used to explore mental problems and preference for support of PAH patients. Additionally, a survey was used to assess the presence of mental problems (Problem List), distress (Distress Thermometer) and the need for mental support. In 24 semi-structured interviews, the following problem themes were identified: energy balance; loss of abilities; feeling misunderstood; and handling of worries and emotions...
July 2017: Pulmonary Circulation
Nikhil Bommakanti, Ayman Isbatan, Avni Bavishi, Gourisree Dharmavaram, Andreia Z Chignalia, Randal O Dull
Hypercapnic acidosis (HCA) has beneficial effects in experimental models of lung injury by attenuating inflammation and decreasing pulmonary edema. However, HCA increases pulmonary vascular pressure that will increase fluid filtration and worsen edema development. To reconcile these disparate effects, we tested the hypothesis that HCA inhibits endothelial mechanotransduction and protects against pressure-dependent increases in the whole lung filtration coefficient (Kf). Isolated perfused rat lung preparation was used to measure whole lung filtration coefficient (Kf) at two levels of left atrial pressure (PLA = 7...
July 2017: Pulmonary Circulation
Jeffrey D Marshall, Maor Sauler, Adriano Tonelli, Youlan Rao, Richard Bucala, Patty J Lee, Wassim H Fares
Macrophage migration inhibitory factor (MIF) and 22 a priori selected biomarkers were measured from pulmonary arterial hypertension (PAH) patients. Significant positive correlations were found between MIF and several angiogenic factors suggesting a possible MIF regulation role in PAH angiogenesis and pathobiology, but simultaneously highlighting the biomarkers profiling complexity in PAH.
July 2017: Pulmonary Circulation
Mariana M Clavé, Nair Y Maeda, Claudia R P Castro, Sergio P Bydlowski, Antonio A Lopes
In patients with Eisenmenger syndrome, life expectancy is usually longer than in patients with other forms of pulmonary arterial hypertension (PAH). We conducted a cohort study in which patients were followed over a long period of time in an attempt to identify potential predictors of clinical outcomes. Sixty-seven treatment-naïve patients were enrolled (age range = 12-60 years; median age = 33 years). Baseline demographic, diagnostic, and functional parameters, plasma levels of endothelial dysfunction markers, and treatment-related data were tested for possible correlations with event-free survival...
July 2017: Pulmonary Circulation
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...
July 2017: Pulmonary Circulation
Jennifer Tingo, Erika B Rosenzweig, Steven Lobritto, Usha S Krishnan
Portopulmonary hypertension (PoPH) is defined by the combination of portal hypertension and precapillary pulmonary arterial hypertension (PAH). Very little is known about this process in pediatric patients but prognosis is generally poor. We review our institutional experience and report on five patients with pediatric PoPH. The median age of PoPH diagnosis was six years and PAH was 14 years. PAH diagnosis was made by echocardiogram in all patients, four of whom also had cardiac catheterization. The median mean pulmonary artery pressure (mPAP) was 48...
July 2017: Pulmonary Circulation
Tamara Roldan, Juan J Rios, Elena Villamañan, Aaron B Waxman
Anticoagulants are widely used in patients with pulmonary arterial hypertension (PAH) to prolong survival. However, there is a lack of robust evidence demonstrating the benefits of anticoagulants in PAH patients and very little is known about the complications of their use in this population. The objective of this study is to compare the safety of routine administration of oral anticoagulants between PAH patients who were and were not treated with oral anticoagulants. This observational, retrospective cohort study included consecutive patients with confirmed PAH from two centers: Brigham and Women's Hospital in Boston and Hospital Universitario La Paz in Madrid from January 2009 to August 2015...
July 2017: Pulmonary Circulation
Amresh Raina, Raymond L Benza, Harrison W Farber
Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-PAH-CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH-CTD include prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH-CTD related to limited scleroderma who were switched from a PDE-5i to riociguat due to insufficient clinical response...
July 2017: Pulmonary Circulation
Franck F Rahaghi, Hassan M Alnuaimat, Rana L A Awdish, Vijay P Balasubramanian, Robert C Bourge, Charles D Burger, John Butler, C Gregory Cauthen, Murali M Chakinala, Bennett P deBoisblanc, Michael S Eggert, Peter Engel, Jeremy Feldman, J Wesley McConnell, Myung Park, Jeffrey S Sager, Namita Sood, Harold I Palevsky
In patients treated with macitentan (Opsumit®, Actelion Pharmaceuticals Ltd., Basel, Switzerland) for pulmonary arterial hypertension (PAH), prevention and/or effective management of treatment-related adverse events may improve adherence. However, management of these adverse events can be challenging and the base of evidence and clinical experience for macitentan is limited. In the absence of evidence, consensus recommendations from physicians experienced in using macitentan to treat PAH may benefit patients and physicians who are using macitentan...
July 2017: Pulmonary Circulation
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...
July 2017: Pulmonary Circulation
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...
July 2017: Pulmonary Circulation
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...
July 2017: Pulmonary Circulation
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