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Pediatric pulmonary hypertension

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https://www.readbyqxmd.com/read/29777282/ambulatory-intravenous-inotropic-support-and-or-levosimendan-in-pediatric-and-congenital-heart-failure-safety-survival-improvement-or-transplantation
#1
Sotiria C Apostolopoulou, George A Vagenakis, Alexandros Tsoutsinos, Felicia Kakava, Spyridon Rammos
End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29767574/right-ventricular-stroke-work-correlates-with-outcomes-in-pediatric-pulmonary-arterial-hypertension
#2
Weiguang Yang, Alison L Marsden, Michelle T Ogawa, Charlotte Sakarovitch, Keeley K Hall, Marlene Rabinovitch, Jeffrey A Feinstein
No abstract text is available yet for this article.
January 1, 2018: Pulmonary Circulation
https://www.readbyqxmd.com/read/29756719/-pulmonary-hypertension-associated-with-the-human-immunodeficiency-virus-in-children-treatment-with-sildenafil-a-case-report
#3
Carolina Stepffer, Evelyn Gaynor, Mariela López, Norma E González, Micaela Arri, Ana Ms De Dios
Pulmonary hypertension associated with human immunodeficiency virus infection is an extremely rare disease in pediatrics; it requires a high clinical suspicion to reach a diagnosis. Its appearance poses an unfavorable prognostic, but early diagnosis and specific treatment can improve outcomes. We report the clinical case of a fifteen-year-old patient diagnosed with human immunodeficiency virus infection of vertical transmission, without antiretroviral treatment, with cough and progressive exertional dyspnea, associated with signs of right heart failure in which severe pulmonary hypertension was diagnosed...
June 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29703037/fatal-malignant-pertussis-with-hyperleukocytosis-in-a-chinese-infant-a-case-report-and-literature-review
#4
Shu-Feng Tian, Hong-Mei Wang, Ji-Kui Deng
RATIONALE: Pertussis has re-emerged on a global scale and is an ongoing public health problem, even in countries with high rates of vaccination. Hyperleukocytosis [white blood cell (WBC) count >100 × 10/L] is a rare complication that strongly predicts mortality in cases of severe pertussis. PATIENT CONCERNS: We report a case of severe pertussis in an infant who initially presented with persistent cyanotic cough, tachypnea, and grunting. The infant's condition deteriorated rapidly, and she was transferred to the pediatric intensive care unit (PICU) during her third hour of hospitalization...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29664507/echocardiographic-evaluation-after-pediatric-heart-transplant-in-chile-initial-application-of-a-functional-protocol-with-global-longitudinal-strain
#5
Claudia Trincado, Víctor Molina, Gonzalo Urcelay, Paulina Dellepiane
INTRODUCTION: The echocardiographic evaluation of patients after heart transplantation is a useful tool. However, it is still necessary to define an optimal follow-up protocol. OBJECTIVE: To describe the results of the application of a functional echocardiographic protocol in patients being followed after pediatric heart transplantation. PATIENTS AND METHOD: Alls patients being followed at our institution after pediatric heart transplantation underwent an echocardiographic examination with a functional protocol that included global longitudinal strain...
February 2018: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29650343/safety-efficacy-and-management-of-subcutaneous-treprostinil-infusions-in-the-treatment-of-severe-pediatric-pulmonary-hypertension
#6
Marilyne Levy, Maria-Jesus Del Cerro, Sophie Nadaud, Karunakar Vadlamudi, Elizabeth Colgazier, Jeff Fineman, Damien Bonnet, Ian Adatia
BACKGROUND: Continuous intravenous epoprostenol was the first treatment approved for pulmonary arterial hypertension (PAH) but administration through a central venous line carries risks of thrombosis and sepsis, particularly in children. We sought to evaluate the safety, efficacy and management of subcutaneous (SC) treprostinil in children with PAH. METHODS: Fifty-six children (median age 65, range 1-200 months) were treated with SC treprostinil. Clinical status, echocardiography, NT-proBNP, and site pain and infection were evaluated...
March 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29628412/the-left-ventricle-in-congenital-diaphragmatic-hernia-implications-for-the-management-of-pulmonary-hypertension
#7
John P Kinsella, Robin H Steinhorn, Mary P Mullen, Rachel K Hopper, Roberta L Keller, D Dunbar Ivy, Eric D Austin, Usha S Krishnan, Erika B Rosenzweig, Jeffrey R Fineman, Allen D Everett, Brian D Hanna, Tilman Humpl, J Usha Raj, Steven H Abman
No abstract text is available yet for this article.
April 5, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29610158/lung-volume-reduction-surgery-for-respiratory-failure-in-infants-with-bronchopulmonary-dysplasia
#8
Bongyeon Sohn, Samina Park, In Kyu Park, Young Tae Kim, June Dong Park, Sung-Hye Park, Chang Hyun Kang
Lung volume reduction surgery (LVRS) can be performed in patients with severe emphysematous disease. However, LVRS in pediatric patients has not yet been reported. Here, we report our experience with 2 cases of pediatric LVRS. The first patient was a preterm infant girl with severe bronchopulmonary dysplasia, pulmonary hypertension, and hypothyroidism. The emphysematous portion of the right lung was removed via sternotomy and right hemiclamshell incision. The patient was discharged on full-time home ventilator support for 3 months after the surgery...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29602613/noninvasive-echocardiographic-measures-of-pulmonary-vascular-resistance-in-children-and-young-adults-with-cardiomyopathy
#9
Dor Markush, Robert D Ross, Ronald Thomas, Sanjeev Aggarwal
BACKGROUND: Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM. METHODS: Imaging studies were retrospectively reviewed from pediatric patients with CM who underwent both echocardiography and cardiac catheterization within a 72-hour period...
March 27, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29596267/a-stewardship-program-to-optimize-the-use-of-inhaled-nitric-oxide-in-pediatric-critical-care
#10
Tanya Di Genova, Christina Sperling, Ashley Gionfriddo, Zelia Da Silva, Leanne Davidson, Jason Macartney, Michael Finelli, Robert P Jankov, Peter C Laussen
PURPOSE: Inhaled nitric oxide (iNO) is a pulmonary vasodilator that is approved for use in term and near-term neonates with hypoxic respiratory failure associated with evidence of pulmonary hypertension. However, it is commonly used in infants and children to treat a variety of other cardiopulmonary diseases associated with pulmonary hypertension and hypoxic respiratory failure. In critically ill children, iNO therapy may be continued for a prolonged period, and this increases the risk for adverse consequences including toxicity and unnecessary costs...
April 2018: Quality Management in Health Care
https://www.readbyqxmd.com/read/29575641/nt-pro-bnp-a-marker-for-worsening-respiratory-status-and-mortality-in-infants-and-young-children-with-pulmonary-hypertension
#11
Shahnawaz M Amdani, Muhammad Umair M Mian, Ron L Thomas, Robert D Ross
AIM: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed. METHODS: A retrospective review of all infants and children < 2 years of age with PH from January 2011 to August 2016 was conducted. Correlations were determined using Spearman's rank correlation coefficients. Differences in characteristics between survivors and nonsurvivors were analyzed and Kaplan-Meier survival curves were generated...
March 25, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29570688/diagnosis-evaluation-and-treatment-of-pulmonary-arterial-hypertension-in-children
#12
REVIEW
Benjamin S Frank, D Dunbar Ivy
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure...
March 23, 2018: Children
https://www.readbyqxmd.com/read/29517668/the-efficiency-of-endothelin-receptor-antagonist-bosentan-for-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-a-systematic-review-and-meta-analysis
#13
REVIEW
Hong-Yu Kuang, Yu-Hao Wu, Qi-Jian Yi, Jie Tian, Chun Wu, We Nian Shou, Tie-Wei Lu
BACKGROUND: Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute responses and a long-term effect were respectively assessed in a comparison with baseline characteristics, and the improvement of exercise tolerance was analyzed. METHODS: PubMed, Medline, Embase, and Cochrane Central Register of clinical controlled trails or observational studies have been searched for a recording of bosentan effects on the PAH-CHD participants...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29505663/the-right-ventricular-outflow-tract-in-pediatric-pulmonary-hypertension-data-from-the-european-pediatric-pulmonary-vascular-disease-network
#14
Martin Koestenberger, Alexander Avian, Hannes Sallmon, Andreas Gamillscheg, Gernot Grangl, Stefan Kurath-Koller, Sabrina Schweintzger, Ante Burmas, Georg Hansmann
OBJECTIVE: The right ventricular outflow tract (RVOT) is pivotal for adequate RV function and known to be adversely affected by elevated pulmonary arterial pressure (PAP) in adults with pulmonary hypertension (PH). Aim of this study was to determine the effects of increased RV pressure afterload in children with PH on RVOT size, function, and flow parameters. METHODS: We conducted a transthoracic echocardiographic study in 51 children with PH (median age: 5.3 years; range 1...
March 5, 2018: Echocardiography
https://www.readbyqxmd.com/read/29498925/dasatinib-in-pediatric-patients-with-chronic-myeloid-leukemia-in-chronic-phase-results-from-a-phase-ii-trial
#15
Lia Gore, Pamela R Kearns, Maria Lucia de Martino, Lee, Carmino Antonio De Souza, Yves Bertrand, Nobuko Hijiya, Linda C Stork, Nack-Gyun Chung, Rocio Cardenas Cardos, Tapan Saikia, Franca Fagioli, Jong Jin Seo, Judith Landman-Parker, Donna Lancaster, Andrew E Place, Karen R Rabin, Mariana Sacchi, Rene Swanink, C Michel Zwaan
Purpose Safe, effective treatments are needed for pediatric patients with chronic myeloid leukemia in chronic phase (CML-CP). Dasatinib is approved for treatment of adults and children with CML-CP. A phase I study determined suitable dosing for children with Philadelphia chromosome-positive (Ph+) leukemias. Methods CA180-226/NCT00777036 is a phase II, open-label, nonrandomized prospective trial of patients < 18 years of age receiving dasatinib. There are three cohorts: (1) imatinib-resistant/intolerant CML-CP, (2) imatinib-resistant/intolerant CML in accelerated/blast phase or Ph+ acute lymphoblastic leukemia (n = 17), and (3) newly diagnosed CML-CP treated with tablets or powder for oral suspension...
May 1, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29496397/effect-of-electrical-dyssynchrony-on-left-and-right-ventricular-mechanics-in-children-with-pulmonary-arterial-hypertension
#16
Michal Schäfer, Kathryn K Collins, Lorna P Browne, D Dunbar Ivy, Steven Abman, Richard Friesen, Benjamin Frank, Brian Fonseca, Michael DiMaria, Kendall S Hunter, Uyen Truong, Johannes C von Alvensleben
BACKGROUND: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony...
February 6, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29490974/pediatric-and-adolescent-pulmonary-hypertension-what-is-the-risk-of-undergoing-invasive-hemodynamic-testing
#17
EDITORIAL
Anudeep Jassal, Omer Cavus, Elisa A Bradley
No abstract text is available yet for this article.
February 28, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29489682/the-clinical-characteristics-and-the-features-of-immunophenotype-of-peripheral-lymphocytes-of-adult-onset-chronic-active-epstein-barr-virus-disease-at-a-tertiary-care-hospital-in-beijing
#18
Ling Luo, Huanling Wang, Hongwei Fan, Jing Xie, Zhifeng Qiu, Taisheng Li
Chronic active Epstein-Barr virus (CAEBV) infection is a rare disease with high mortality. Most of CAEBV patients have been reported from Japan and are pediatric cases.The goal was to describe the clinical characteristics and the immunophenotypic features of peripheral lymphocytes in adult onset CAEBV patients.We retrospectively reviewed and analyzed all adult onset CAEBV cases admitted to Peking Union Medical College Hospital (PUMCH) between 2012 and 2016. Demographic, clinical, laboratory data, and the immunophentyping data of peripheral lymphocytes were collected...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29486304/development-of-a-portable-mini-generator-to-safely-produce-nitric-oxide-for-the-treatment-of-infants-with-pulmonary-hypertension
#19
Binglan Yu, Michele Ferrari, Grigorij Schleifer, Aron H Blaesi, Martin Wepler, Warren M Zapol, Donald B Bloch
OBJECTIVES: To test the safety of a novel miniaturized device that produces nitric oxide (NO) from air by pulsed electrical discharge, and to demonstrate that the generated NO can be used to vasodilate the pulmonary vasculature in rabbits with chemically-induced pulmonary hypertension. STUDY DESIGN: A miniature NO (mini-NO) generator was tested for its ability to produce therapeutic levels (20-80 parts per million (ppm)) of NO, while removing potentially toxic gases and metal particles...
May 1, 2018: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/29480089/right-ventricular-function-mirrors-clinical-improvement-with-use-of-prostacyclin-analogues-in-pediatric-pulmonary-hypertension
#20
Rachel K Hopper, Yan Wang, Valerie DeMatteo, Ashley Santo, Steven M Kawut, Okan U Elci, Brian D Hanna, Laura Mercer-Rosa
Pulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children with PH treated with a prostacyclin analogue (epoprostenol or treprostinil) between January 2001 and August 2015 at our center. Data were collected before initiation of treatment (baseline) and at 1-3 and 6-12 months after...
April 2018: Pulmonary Circulation
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