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Pediatric pulmonary hypertension

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https://www.readbyqxmd.com/read/28193613/apparent-aortic-stiffness-in-children-with-pulmonary-arterial-hypertension-existence-of-vascular-interdependency
#1
Michal Schäfer, D Dunbar Ivy, Steven H Abman, Alex J Barker, Lorna P Browne, Brian Fonseca, Vitaly Kheyfets, Kendall S Hunter, Uyen Truong
BACKGROUND: Left ventricular dysfunction, mediated by ventricular interdependence, has been associated with negative outcomes in children with pulmonary arterial hypertension (PAH). Considering the dilation of the pulmonary arteries as a paramount sign of PAH, we hypothesized that the ascending aorta will present signs of apparent stiffness in children with PAH and that this effect may be because of mechanical interaction with the dilated main pulmonary artery (MPA). METHODS AND RESULTS: Forty-two children with PAH and 26 age- and size-matched controls underwent comprehensive cardiac magnetic resonance evaluation...
February 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28185814/obstructive-sleep-apnea-and-pulmonary-hypertension-in-children
#2
REVIEW
David G Ingram, Alvin V Singh, Zarmina Ehsan, Brian F Birnbaum
Obstructive sleep apnea (OSA) is a common pediatric breathing disorder, affecting 1-5% of all children. Pulmonary hypertension (PH), a severe complication of OSA, is associated with significant morbidity and mortality. Despite this important relationship between OSA and PH, there is sparse literature addressing this subject in children. This review will examine the putative relationship between OSA and PH, synthesize the available literature in children, and suggest a reasonable approach, despite limited data, for clinicians...
January 11, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28185800/inhaled-sildenafil-as-an-alternative-to-oral-sildenafil-in-the-treatment-of-pulmonary-arterial-hypertension-pah
#3
Jahidur Rashid, Brijeshkumar Patel, Eva Nozik-Grayck, Ivan F McMurtry, Kurt R Stenmark, Fakhrul Ahsan
The practice of treating PAH patients with oral or intravenous sildenafil suffers from the limitations of short dosing intervals, peripheral vasodilation, unwanted side effects, and restricted use in pediatric patients. In this study, we sought to test the hypothesis that inhalable poly(lactic-co-glycolic acid) (PLGA) particles of sildenafil prolong the release of the drug,produce pulmonary specific vasodilation, reduce the systemic exposure of the drug, and may be used as an alternative to oral sildenafil in the treatment of PAH...
February 6, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28178426/physical-activity-in-pediatric-pulmonary-arterial-hypertension-measured-by-accelerometry-a-candidate-clinical-endpoint
#4
Willemijn M H Zijlstra, Mark-Jan Ploegstra, Theresia Vissia-Kazemier, Marcus T R Roofthooft, Gideon du Marchie Sarvaas, Beatrijs Bartelds, Annette Rackowitz, Freek van den Heuvel, Hans L Hillege, Guy Plasqui, Rolf M F Berger
Rationale- The development of evidence-based treatment guidelines for pediatric pulmonary arterial hypertension (PAH) is hampered by lack of pediatric clinical trials. Trial-design is hampered by lack of a feasible clinical endpoint in this population. Objectives- To evaluate the use of accelerometry for measuring physical activity (PA) in pediatric PAH and to investigate its correlation with clinical disease severity markers. Methods- We included children from the Dutch National Network for Pediatric Pulmonary Hypertension...
February 8, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28145758/serially-measured-uric-acid-levels-predict-disease-severity-and-outcome-in-pediatric-pulmonary-arterial-hypertension
#5
Lynn J Leberkühne, Mark-Jan Ploegstra, Johannes M Douwes, Beatrijs Bartelds, Marcus T R Roofthooft, Hans L Hillege, Rolf M F Berger
No abstract text is available yet for this article.
February 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#6
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
https://www.readbyqxmd.com/read/28105408/predictors-of-prolonged-mechanical-ventilation-in-pediatric-patients-after-cardiac-surgery-for-congenital-heart-disease
#7
Avisa Tabib, Seyed Ehsan Abrishami, Mohammad Mahdavi, Hojjat Mortezaeian, Ziae Totonchi
BACKGROUND: The duration of mechanical ventilation (MV) is one of the most important clinical factors which predict outcomes in pediatric cardiac surgery. The prolonged mechanical ventilation (PMV) following cardiac surgery is a multifactorial phenomenon and there are conflicts regarding its predictors in pediatric population between different centers. OBJECTIVES: The current study aimed to describe PMV predictors in patients undergoing cardiac surgery for congenital heart disease in a tertiary center for pediatric cardiovascular diseases in Iran...
August 2016: Research in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28103851/cystic-fibrosis-physicians-perspectives-on-the-timing-of-referral-for-lung-transplant-evaluation-a-survey-of-physicians-in-the-united-states
#8
Kathleen J Ramos, Ranjani Somayaji, Erika D Lease, Christopher H Goss, Moira L Aitken
BACKGROUND: Prior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician perspectives on the timing of LTx referral and investigate their LTx knowledge. METHODS: We developed an online anonymous survey that was distributed by the Cystic Fibrosis Foundation (CFF) to the medical directors of all CFF-accredited care centers in the United States in 2015...
January 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28101803/lung-retransplantation-in-an-adult-13%C3%A2-years-after-single-lobar-transplant-in-childhood
#9
Seiichiro Sugimoto, Shinji Otani, Takashi Ohki, Takeshi Kurosaki, Kentaroh Miyoshi, Masaomi Yamane, Shinichiro Miyoshi, Takahiro Oto
Single living-donor lobar lung transplantation provides acceptable results for critically ill children; however, an additional lung transplantation may be required in the future as the recipient grows. We describe a case of successful lung retransplantation in a grown-up patient after single lobar lung transplantation in childhood. A 23-year-old man underwent bilateral cadaveric lung retransplantation for chronic lung allograft dysfunction 13 years after right single living-donor lobar transplantation for idiopathic pulmonary arterial hypertension performed at the age of 10 years...
January 18, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28094679/a-retrospective-analysis-of-a-pediatric-tele-echocardiography-service-to-treat-triage-and-reduce-trans-pacific-transport
#10
Christopher A Rouse, Brandon T Woods, C Becket Mahnke
Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#11
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/28078552/pediatric-development-of-bosentan-facilitated-by-modeling-and-simulation
#12
Jochen Zisowsky, Martine Géhin, Andjela Kusic-Pajic, Andreas Krause, Maurice Beghetti, Jasper Dingemanse
BACKGROUND: Bosentan is approved for use in adult patients with pulmonary arterial hypertension. The primary aim of the pharmacokinetic modeling was the provision of a systematic guidance for study design and enhanced understanding of pharmacokinetics across the entire pediatric age range. METHODS: A physiologically based pharmacokinetic model was developed for the pediatric population; starting from an adult model, the effects of body weight, age, and maturation of relevant metabolizing enzymes were incorporated to extrapolate the pharmacokinetics to children...
January 11, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28072955/-clinical-analysis-of-12-patients-with-pediatric-antiphospholipid-syndrome-with-pulmonary-embolism
#13
J R Ma, H M Song, J Xiao, X Y Tang, Y Y He, M Wei
Objective: To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism. Method: Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism. Result: Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28072745/safety-and-efficacy-of-chloral-hydrate-for-conscious-sedation-of-infants-in-the-pediatric-cardiovascular-intensive-care-unit
#14
Mei-Lian Chen, Qiang Chen, Fan Xu, Jia-Xin Zhang, Xiao-Ying Su, Xiao-Zhen Tu
This study evaluates the safety and efficacy of chloral hydrate administration for the conscious sedation of infants in the pediatric cardiovascular intensive care unit (PCICU).We conducted a retrospective review of the charts of 165 infants with congenital heart disease who received chloral hydrate in our PCICU between January 2014 and December 2014. Chloral hydrate was administered orally or rectally to infants using doses of 50 mg/kg. We collected and analyzed relevant clinical parameters.The overall length of time to achieve sedation was ranged from 5 to 35 min (10...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28060126/pediatric-acquired-von-willebrand-syndrome-in-cardiopulmonary-disorders-do-laboratory-abnormalities-predict-bleeding-risk
#15
Saman K Hashmi, Mireya P Velasquez, Donald L Yee, Shiu-Ki Hui, Donald Mahoney, Lakshmi V Srivaths
There are conflicting reports on whether or not laboratory abnormalities in pediatric acquired von Willebrand syndrome (AVWS) predict bleeding manifestations in patients with cardiopulmonary disorders (CPD). We retrospectively reviewed charts of patients with AVWS and CPD (n=16) seen at Texas Children's Hospital from 2003 to 2012. The most common CPD were valve stenoses, ventricular septal defects, and pulmonary hypertension. All patients had loss of high molecular weight multimers. Fifteen (94%) patients presented with bleeding symptoms, with menorrhagia and epistaxis being the most common...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28029743/statins-ameliorate-pulmonary-hypertension-secondary-to-left-ventricular-dysfunction-through-the-rho-kinase-pathway-and-nadph-oxidase
#16
I-Chen Chen, Mian-Shin Tan, Bin-Nan Wu, Chee-Yin Chai, Jwu-Lai Yeh, Shah-Hwa Chou, Ing-Jun Chen, Zen-Kong Dai
BACKGROUND: Pulmonary hypertension (PH) is a devastating disorder, for which no therapy is curative. It has been reported that pulmonary vascular remodeling, associated with increasing mean pulmonary arterial pressure and upregulated expression of endothelial nitric oxide synthase (eNOS), endothelin-1 (ET-1), RhoA/RhoH-kinase results in the development of PH. Oxidative stress and the RhoA/Rho-kinase pathway are also thought to be involved in the pathophysiology of PH. Statins are 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (HMG-CoA reductase inhibitors) with pleiotropic effects and are potential agents for the treatment of PH...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27995825/treatment-initiation-in-paediatric-pulmonary-hypertension-insights-from-a-multinational-registry
#17
Tilman Humpl, Rolf M F Berger, Eric D Austin, Margrit S Fasnacht Boillat, Damien Bonnet, Dunbar D Ivy, Malgorzata Zuk, Maurice Beghetti, Ingram Schulze-Neick
Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry. Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected...
December 20, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27988223/mexican-registry-of-pulmonary-hypertension-remehip
#18
Julio Sandoval Zarate, Carlos Jerjes-Sanchez, Alicia Ramirez-Rivera, Tomas Pulido Zamudio, Pedro Gutierrez-Fajardo, Jose Elizalde Gonzalez, Mario Seoane Garcia De Leon, Miguel Beltran Gamez, Francisco Moreno Hoyos Abril, Rodolfo Parra Michel, Humberto Garcia Aguilar
OBJECTIVE: REMEHIP is a prospective, multicentre registry on pulmonary hypertension. The main objective will be to identify the clinical profile, medical care, therapeutic trends and outcomes in adult and pediatric Mexican patients with well-characterized pulmonary hypertension. METHODS: REMEHIP a multicenter registry began in 2015 with a planned recruitment time of 12 months and a 4-year follow-up. The study population will comprise a longitudinal cohort study, collecting data on patients with prevalent and incident pulmonary hypertension...
January 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/27925367/transient-severe-tricuspid-regurgitation-after-transplantation-of-an-extremely-oversized-donor-heart-in-a-child-does-size-matter-a-case-report
#19
J Birnbaum, S M Ulrich, R Schramm, C Hagl, A Lehner, M Fischer, N A Haas, B Heineking
In pediatric heart transplantation, the size of the donor organ is an important criterion for organ allocation. Oversized donor hearts are often accepted with good results, but some complications in relation to a high donor-recipient ratio have been described. Our patient was transplanted for progressive heart failure in dilated cardiomyopathy. The donor-to-recipient weight ratio was 3 (donor weight 65 kg, recipient weight 22 kg). The intra-operative echocardiography before chest closure showed excellent cardiac function, no tricuspid valve regurgitation, and a normal central venous pressure...
February 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/27877095/evaluation-of-the-tolerability-of-intermittent-intravenous-sildenafil-in-pediatric-patients-with-pulmonary-hypertension
#20
Robyn A Fender, Ty E Hasselman, Yanzhi Wang, Aaron A Harthan
OBJECTIVES: The primary purpose of this study was to determine the tolerability of intermittent intravenous (IV) sildenafil for the treatment of pulmonary hypertension in pediatric patients. Secondary objectives were to evaluate parameters related to efficacy. METHODS: This was a retrospective chart review from January 2013 to August 2014 of pediatric patients under age 18 years treated with intermittent doses of IV sildenafil for pulmonary hypertension. Patients were excluded if they were over age 18 years or received sildenafil for other indications...
September 2016: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
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