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Pediatric pulmonary arterial hypertension

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https://www.readbyqxmd.com/read/27861431/pediatric-artificial-lung-a-low-resistance-pumpless-artificial-lung-alleviates-an-acute-lamb-model-of-increased-right-ventricle-afterload
#1
Fares Alghanem, Benjamin S Bryner, Emilia M Jahangir, Uditha P Fernando, John M Trahanas, Hayley R Hoffman, Robert H Bartlett, Alvaro Rojas-Peña, Ronald B Hirschl
Lung disease in children often results in pulmonary hypertension and right heart failure. The availability of a pediatric artificial lung (PAL) would open new approaches to the management of these conditions by bridging to recovery in acute disease or transplantation in chronic disease. This study investigates the efficacy of a novel PAL in alleviating an animal model of pulmonary hypertension and increased right ventricle afterload.Five juvenile lambs (20-30 Kg) underwent PAL implantation in a pulmonary artery to left atrium configuration...
November 15, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27860161/closure-of-very-large-pda-with-pulmonary-hypertension-initial-clinical-case-series-with-the-new-occlutech-%C3%A2-pda-occluder
#2
Anja Lehner, Sarah Ulrich, Christoph M Happel, Marcus Fischer, Marinos Kantzis, Ingram Schulze-Neick, Nikolaus A Haas
OBJECTIVE: We report our recent experience with the new Occlutech(®) PDA Occluder, which seems suitable especially for closure of large patent ductus arteriosus (PDA) sizes with elevated pulmonary artery pressure (PAH). BACKGROUND: Although considered a standard procedure in pediatric cath labs, closure of very large PDA (PDA/Aorta ratio > 0.5) and PAH remains a challenge. METHODS/RESULTS: The Occlutech(®) PDA occluder was used in eight children and adolescents (age 4-16, median 10...
November 10, 2016: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/27832003/development-of-a-model-of-pediatric-lung-failure-pathophysiology
#3
John M Trahanas, Fares Alghanem, Catalina Ceballos-Muriel, Hayley R Hoffman, Alice Xu, Kristopher B Deatrick, Marie Cornell, Alvaro Rojas-Pena, Robert H Bartlett, Ronald B Hirschl
A pediatric artificial lung (PAL) is under development as a bridge to transplantation or lung remodeling for children with end-stage lung failure (ESLF). In order to evaluate the efficiency of a PAL, a disease model mimicking the physiologic derangements of pediatric ESLF is needed. Our previous right pulmonary artery ligation (rPA-LM) ovine model achieved that goal, but caused immediate mortality in nearly half of the animals. In this study, we evaluated a new technique of gradual postoperative right pulmonary artery (rPA) occlusion using a Rummel tourniquet (rPA-RT) in seven (25-40 Kg) sheep...
November 9, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27826712/subclinical-pulmonary-hypertension-in-childhood-systemic-lupus-erythematosus-associated-with-minor-disease-manifestations
#4
Pedro Anuardo, Monica Verdier, Natali W S Gormezano, Gabriela R V Ferreira, Gabriela N Leal, Alessandro Lianza, Juliana C O A Ferreira, Rosa M R Pereira, Nadia E Aikawa, Maria Teresa Terreri, Claudia S Magalhães, Simone Appenzeller, Maria Carolina Dos Santos, Silvana B Sachetti, Claudio A Len, Gecilmara S Pilleggi, Simone Lotufo, Eloisa Bonfá, Clovis A Silva
The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of São Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure >35 mmHg and/or measurement of the mean pulmonary artery pressure >25 mmHg and/or diastolic pressure >15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated...
November 8, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27801982/risk-factors-for-pulmonary-arterial-hypertension-in-children-and-young-adults
#5
Estelle Naumburg, Lars Söderström, Daniel Huber, Inge Axelsson
OBJECTIVES: Pulmonary hypertension (PH) has been linked to preterm birth explained by congenital heart defects and pulmonary diseases. WORKING HYPOTHESIS: Other factors may influence the risk of PH among adolescences and children born premature. STUDY DESIGN: This national registry-based study assess risk of PH following premature birth adjusted for known risk factors. PATIENT-SUBJECT SELECTION AND METHODOLOGY: All cases born 1993-2010, identified by diagnostic codes applicable to PH and retrieved from the Swedish Registry of Congenital Heart Disease (N = 67)...
November 1, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27800456/successful-lung-transplant-after-prolonged-extracorporeal-membrane-oxygenation-ecmo-in-a-child-with-pulmonary-hypertension-a-case-report
#6
Cecile Tissot, Walid Habre, Paola Soccal, Maja Isabel Hug, Dominique Bettex, Michel Pellegrini, Yacine Aggoun, Anne Mornand, Afksendyios Kalangos, Peter Rimensberger, Maurice Beghetti
INTRODUCTION: The use of extracorporeal membrane oxygenation (ECMO) is considered a risk factor for, or even a potential contraindication to, lung transplantation. However, only a few pediatric cases have been described thus far. CASE PRESENTATION: A 9-year-old boy with idiopathic pulmonary arterial hypertension developed cardiac arrest after the insertion of a central catheter. ECMO was used as a bridge to lung transplantation. However, after prolonged resuscitation, he developed medullary ischemia and medullary syndrome...
August 2016: Research in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27770446/the-role-of-genetics-in-pulmonary-arterial-hypertension
#7
REVIEW
Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically, it has been associated with a high mortality rate, although, over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
October 22, 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27760452/-chronic-thromboembolic-pulmonary-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#8
H Wilkens, S Konstantinides, I Lang, A C Bunck, M Gerges, F Gerhardt, A Grgic, C Grohé, S Guth, M Held, J Hinrichs, M M Hoeper, W Klepetko, T Kramm, U Krüger, M Lankeit, B C Meyer, K M Olsson, H-J Schäfers, M Schmidt, H J Seyfarth, S Ulrich, C B Wiedenroth, E Mayer
The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH), but also other significant subgroups of pulmonary hypertension (PH). In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany to discuss open and controversial issues surrounding the practical implementation of the European Guidelines...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760451/-pulmonary-hypertension-due-to-chronic-lung-disease-recommendations-of-the-cologne-consensus-conference-2016
#9
H Olschewski, J Behr, H Bremer, M Claussen, P Douschan, M Halank, M Held, M M Hoeper, S Holt, H Klose, S Krüger, T J Lange, F Reichenberger, D Skowasch, S Ulrich, H Wilkens, W Seeger
The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) associated with chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, sGC stimulators) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760450/-pulmonary-hypertension-associated-with-left-heart-disease-recommendations-of-the-cologne-consensus-conference-2016
#10
S Rosenkranz, I M Lang, R Blindt, D Bonderman, L Bruch, G P Diller, R Felgendreher, C Gerges, W Hohenforst-Schmidt, S Holt, C Jung, I Kindermann, T Kramer, W M Kübler, V Mitrovic, A Riedel, A Rieth, A Schmeisser, R Wachter, J Weil, C Opitz
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. While the guidelines contain detailed recommendations regarding pulmonary arterial hypertension (PAH), they contain only a relatively short paragraph on other, much more common forms of PH such as PH due to left heart disease. Despite the lack of data, targeted PAH treatments are increasingly being used for PH associated with left heart disease. This development is of concern because of limited ressources and the need to base treatments on scientific evidence...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760449/-decompensated-right-heart-failure-intensive-care-and-perioperative-management-in-patients-with-pulmonary-hypertension
#11
K M Olsson, M Halank, B Egenlauf, D Fistera, H Gall, C Kaehler, K Kortmann, T Kramm, M Lichtblau, A Marra, C Nagel, A Sablotzki, H-J Seyfarth, D Schranz, S Ulrich, M M Hoeper, T J Lange
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760448/-targeted-therapy-of-pulmonary-arterial-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#12
M M Hoeper, C Apitz, E Grünig, M Halank, R Ewert, H Kaemmerer, H-J Kabitz, C Kähler, H Klose, H Leuchte, S Ulrich, K M Olsson, O Distler, S Rosenkranz, H A Ghofrani
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760447/-general-and-supportive-therapy-of-pulmonary-arterial-hypertension
#13
E Grünig, N Benjamin, U Krüger, H Kaemmerer, S Harutyunova, K M Olsson, S Ulrich, F Gerhardt, C Neurohr, A Sablotzki, M Halank, H-J Kabitz, G Thimm, K-G Fliegel, H Klose
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted and supportive treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760446/-risk-stratification-and-follow-up-assessment-of-patients-with-pulmonary-arterial-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#14
H H Leuchte, H Ten Freyhaus, H Gall, M Halank, M M Hoeper, H Kaemmerer, C Kähler, G Riemekasten, S Ulrich, M Schwaiblmair, R Ewert
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed information about the diagnosis of pulmonary hypertension, and furthermore provide novel recommendations for risk stratification and follow-up assessments. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760444/-pathobiology-pathology-and-genetics-of-pulmonary-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#15
A Olschewski, E M Berghausen, C A Eichstaedt, B K Fleischmann, E Grünig, G Grünig, G Hansmann, L Harbaum, J K Hennigs, D Jonigk, W M Kübler, G Kwapiszewska, S S Pullamsetti, E Stacher, N Weissmann, D Wenzel, R T Schermuly
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) are also valid for Germany. While the guidelines contain detailed recommendations regarding clinical aspects of pulmonary arterial hypertension (PAH) and other forms of PH, they contain only a relatively short paragraph on novel findings on the pathobiology, pathology, and genetics. However, these are of great importance for our understanding of this complex disease both from a clinical and scientific point of view, and they are essential for the development of novel treatment strategies...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27729606/-pediatric-iatrogenic-vertebral-arteriovenous-fistula-successfully-treated-with-endovascular-treatment-a-case-report
#16
Rintaro Yokoyama, Satoshi Iihoshi, Kei Miyata, Kentaro Toyama, Katsuya Komatsu, Masahiko Wanibuchi, Nobuhiro Mikuni
OBJECT: We report a relatively rare case of pediatric iatrogenic extradural vertebral arteriovenous fistula. CASE: A five-year-old boy with a history of multiple surgeries for Total Anomalous Pulmonary Venous Return(TAPVR)exhibited vascular engorgement of his right neck from about 4 years old. We thought that this was caused by his past operations. He demonstrated swollen blood vessels in the right neck and pulmonary hypertension with increased right heart load...
October 2016: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/27684888/a-0-d-model-and-protocol-for-simulating-patient-specific-pulmonary-hemodynamics-from-limited-clinical-data
#17
Vitaly O Kheyfets, Jamie Dunning, Uyen Truong, Dunbar Ivy, Kendall Hunter, Robin Shandas
In Pulmonary Hypertension (PH) diagnosis and management, many useful functional markers have been proposed that are unfeasible for clinical implementation. In addition to clinical applications, many research projects are hampered by limited retrospective clinical data. The objective of this study was to develop and validate a 0-D computational model, along with an implementation protocol, of the right ventricular-pulmonary artery (RV-PA) axis. The model is validated against pediatric clinical data (N = 111) and utilized to perform a multi-variate statistical study of the complex interplay between functional phenotype and resulting hemodynamics...
September 29, 2016: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/27683607/clinical-classification-in-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#18
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/27635273/reversible-severe-pulmonary-hypertension-after-adenotonsillectomy-a-case-report-of-a-child-treated-at-bugando-medical-centre-northwestern-tanzania
#19
Rogatus Kabyemera, Neema Chami, Neema Kayange, Respicius Bakalemwa, Antke Zuechner, Tumaini Mhada, Gustave Buname, Adolfine Hokororo, Johannes Kataraihya
Upper airway obstruction (UAO) due to adenotonsillar hypertrophy represents one of the rare causes of pulmonary hypertension in children. We report a case of adenotonsillar hypertrophy, managed at pediatric and otorhinolaryngology departments in Bugando Medical Centre (BMC), northwestern Tanzania, with complete remission of symptoms of pulmonary hypertension following adenotonsillectomy. A 17-month-old boy presented with difficulty breathing, dry cough, and noisy breathing since 1 year. He had facial and lower limb oedema with a pan systolic murmur at the tricuspid area, fine crepitations, and tender hepatomegaly...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27623401/corrigendum-to-future-2-results-from-an-open-label-long-term-safety-and-tolerability-extension-study-using-the-pediatric-formulation-of-bosentan-in-pulmonary-arterial-hypertension-int-j-cardiol-202-2016-52-58
#20
Rolf M F Berger, Sheila G Haworth, Damien Bonnet, Yves Dulac, Alain Fraisse, Nazzareno Galiè, D Dunbar Ivy, Xavier Jaïs, Oliver Miera, Erika B Rosenzweig, Michela Efficace, Andjela Kusic-Pajic, Maurice Beghetti
No abstract text is available yet for this article.
November 15, 2016: International Journal of Cardiology
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