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Pediatric pulmonary arterial hypertension

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https://www.readbyqxmd.com/read/29119033/the-utility-of-mri-in-the-diagnosis-of-takayasu-arteritis
#1
Marian Gaballah, Rachelle Goldfisher, John B Amodio
Takayasu Arteritis (TA) is an inflammatory disorder involving the thoracoabdominal aorta and its branches and the pulmonary arteries, with eventual vascular stenosis, occlusion, or aneurysm formation. Conventional angiography has been the reference imaging standard for diagnosis of TA. The purpose of this case report is to demonstrate the utility of MR imaging and MR angiography in the diagnosis of Takayasu Arteritis in a pediatric patient. The patient is a 15-year-old female patient presenting with anemia, hypertension, and acute kidney injury...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/29071783/age-is-not-a-good-predictor-of-irreversibility-of-pulmonary-hypertension-in-congenital-cardiac-malformations-with-left-to-right-shunt
#2
Amir-Reza Hosseinpour, Marie-Hélène Perez, David Longchamp, Jacques Cotting, Nicole Sekarski, Michel Hurni, René Prêtre, Stefano Di Bernardo
OBJECTIVE: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here. DESIGN: A retrospective observational study...
October 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29071163/resolution-of-periodic-breathing-in-a-child-with-idiopathic-pulmonary-arterial-hypertension
#3
Saadoun Bin-Hasan, Abdullah Khayat, Tilman Humpl, Janette T Reyes, Suhail Al-Saleh
Central sleep apnea (CSA) and periodic breathing are unusual findings described in pediatric patients with congestive heart failure. However, CSA has not been reported in children with pulmonary hypertension. We hereby report on a 10-year-old girl with idiopathic pulmonary arterial hypertension who had frequent central events in a periodic breathing fashion seen in her polysomnography, which was normalized following medical treatment leading to improvement of the pulmonary pressures.
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/29064445/pediatric-perioperative-pulmonary-arterial-hypertension-a-case-based-primer
#4
REVIEW
Shilpa Shah, Jacqueline R Szmuszkovicz
The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this unique group of children.
October 24, 2017: Children
https://www.readbyqxmd.com/read/29045954/-pulmonary-arterial-hypertension-as-leading-manifestation-of-methylmalonic-aciduria-clinical-characteristics-and-gene-testing-in-15-cases
#5
X Q Liu, H Yan, J X Qiu, C Y Zhang, J G Qi, X Zhang, H J Xiao, Y L Yang, Y H Chen, J B DU
OBJECTIVE: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation. METHODS: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29043395/measuring-flow-hemodynamic-indices-and-oxygen-consumption-in-children-with-pulmonary-hypertension-a-comparison-of-catheterization-and-phase-contrast-mri
#6
Michal Schäfer, Uyen Truong, Lorna P Browne, Gareth J Morgan, Michael Ross, Richard Ing, Kendall S Hunter, Vitaly O Kheyfets, Steven H Abman, D Dunbar Ivy, Neil Wilson
We sought to compare pulmonary flow hemodynamic indices obtained by Fick and thermodilution catheterization techniques with phase-contrast MRI (PC-MRI) in children with diverse etiologies of pulmonary arterial hypertension (PAH). Calculation of pulmonary flow ([Formula: see text]) using the Fick principle in most catheter laboratories relies on an estimate of oxygen consumption which may limit its reliability. Flow hemodynamic indices acquired from thirty patients with PAH who underwent successful same-day PC-MRI and catheterization were evaluated for absolute and percent bias...
October 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28980052/right-ventricular-tissue-doppler-myocardial-performance-index-in-children-with-pulmonary-hypertension-relation-to-invasive-hemodynamics
#7
Richard M Friesen, Michal Schäfer, Dale A Burkett, Courtney J Cassidy, D Dunbar Ivy, Pei-Ni Jone
Right ventricular (RV) failure is a significant cause of morbidity and mortality in patients with pulmonary hypertension (PH). Myocardial performance index measured by tissue Doppler imaging (TDI-MPI) has been useful in assessing RV dysfunction in adults with PH. However, TDI-MPI as a marker for RV dysfunction or disease severity has not been evaluated in pediatric PH. The aim of this study was to investigate TDI-MPI and correlate with invasive hemodynamics in pediatric PH patients. Eighty pediatric PH patients undergoing cardiac catheterization and simultaneous transthoracic echocardiography were analyzed...
October 4, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28977199/risk-factors-for-delayed-extubation-after-ventricular-septal-defect-closure-a-prospective-observational-study
#8
Divyakant Parmar, Ketav Lakhia, Pankaj Garg, Kartik Patel, Ritesh Shah, Jigar Surti, Jigar Panchal, Himani Pandya
OBJECTIVE: The objective of our study was to determine the feasibility of early extubation and to identify the risk factors for delayed extubation in pediatric patients operated for ventricular septal defect closure. METHODS: A prospective, observational study was carried out at our Institute. This study involved consecutive 135 patients undergoing ventricular septal defect closure. Patients were extubated if feasible within six hours after surgery. Based on duration of extubation, patients were divided two groups: Group 1= extubation time ≤ 6 hours, Group 2= extubation time >6 hours...
July 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28904266/clinical-impact-of-main-pulmonary-artery-dilatation-on-outcome-in-pediatric-idiopathic-and-heritable-pulmonary-arterial-hypertension
#9
Satoshi Ikehara, Shinichi Takatsuki, Tomotaka Nakayama, Kazuyuki Naoi, Hiroyuki Matsuura, Tsutomu Saji
BACKGROUND: Few studies have investigated the clinical impact of pulmonary artery (PA) dilatation on outcomes in pediatric pulmonary arterial hypertension (PAH).Methods and Results:This study investigated the clinical outcomes of idiopathic or heritable PAH in 66 children aged <18 years at diagnosis. Main PA/thorax (MPA/T) ratio was measured on chest radiography in PAH patients. Patients were divided into 2 groups based on MPA/T ratio, and compared with a control group of 166 age- and gender-matched healthy children...
September 13, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28900614/echocardiographic-evaluation-of-pulmonary-pressures-and-right-ventricular-function-after-pediatric-cardiac-surgery-a-simple-approach-for-the-intensivist
#10
REVIEW
Maurice Beghetti
Pulmonary hypertension (PH) is diagnosed using cardiac catheterization and is defined as an elevation of mean pulmonary artery pressure (PAP) greater than 25 mmHg. Although invasive hemodynamics remains the gold standard and is mandatory for disease confirmation, transthoracic echocardiography (TTE) is an extremely useful non-invasive and widely available tool that allows for screening and follow-up, in particular, in the acute setting. TTE may be a valuable alternative, allowing for direct measurement and/or indirect assessment of PAP...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28873355/pulmonary-interstitial-glycogenosis-associated-with-a-spectrum-of-neonatal-pulmonary-disorders
#11
Ernest Cutz, Rose Chami, Sharon Dell, Jacob Langer, David Manson
Primary or isolated pulmonary interstitial glycogenosis (PIG) is a rare disease presenting as tachypnea and hypoxemia during the perinatal period. A diffuse interstitial infiltrate with focal hyperinflation is visible on chest imaging. The biopsy findings include diffuse expansion of the interstitium by spindle-shaped cells with pale cytoplasm that, on electron microscopy (EM), are poorly differentiated mesenchymal cells containing abundant monoparticulate glycogen. This glycogenosis appears to be a transient abnormality, usually with a favorable prognosis...
September 2, 2017: Human Pathology
https://www.readbyqxmd.com/read/28832796/transverse-sternal-split-a-safe-mini-invasive-approach-for-perventricular-device-closure-of-ventricular-septal-defect
#12
Pankaj Garg, Arvind Kumar Bishnoi, Ketav Lakhia, Jigar Surti, Sumbul Siddiqui, Parth Solanki, Himani Pandya
Objective: Perventricular device closure of ventricular septal defect through midline sternotomy avoids the cardiopulmonary bypass, however, lacks the cosmetic advantage. Perventricular device closure of ventricular septal defect with transverse split sternotomy was performed to add the cosmetic advantage of mini-invasive technique. Methods: Thirty-six pediatric patients with mean age 7.14±3.24 months and weight 5.00±0.88 kg were operated for perventricular device closure of ventricular septal defect through transverse split sternotomy in 4th intercostal space under transesophageal echocardiography guidance...
May 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28819713/plasma-growth-differentiation-factor-15-is-a-potential-biomarker-for-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#13
Gang Li, Yan Li, Xiao-Qiu Tan, Peng Jia, Jian Zhao, Dong Liu, Ting Wang, Bin Liu
We aimed to investigate plasma growth differentiation factor-15 (GDF-15) levels in pediatric pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD), and assess the association with hemodynamic parameters. Plasma GDF-15 levels were measured in children with PAH-CHD (n = 46) and compared to children with CHD without PAH (n = 39). Normal individuals (n = 30) served as health control group. Plasma GDF-15 levels were significantly elevated in patients with PAH-CHD compared with those with CHD without PAH (median 1415 ng/L, interquartile range [IQR] 926...
August 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28819545/circulating-mirnas-in-pediatric-pulmonary-hypertension-show-promise-as-biomarkers-of-vascular-function
#14
Vitaly O Kheyfets, Carmen C Sucharov, Uyen Truong, Jamie Dunning, Kendall Hunter, Dunbar Ivy, Shelley Miyamoto, Robin Shandas
BACKGROUND/OBJECTIVES: The objective of this study was to evaluate the utility of circulating miRNAs as biomarkers of vascular function in pediatric pulmonary hypertension. METHOD: Fourteen pediatric pulmonary arterial hypertension patients underwent simultaneous right heart catheterization (RHC) and blood biochemical analysis. Univariate and stepwise multivariate linear regression was used to identify and correlate measures of reactive and resistive afterload with circulating miRNA levels...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28791664/pharmacokinetics-of-sildenafil-in-children-with-pulmonary-arterial-hypertension
#15
Hugo Juárez Olguín, Hector Osnaya Martínez, Carmen Flores Pérez, Blanca Ramírez Mendiola, Liliana Rivera Espinosa, Juan Luis Chávez Pacheco, Janett Flores Pérez, Ignacio Mora Magaña
BACKGROUND: Recently, sildenafil was introduced to treat pulmonary arterial hypertension (PAH); however, there are currently few studies on the pharmacokinetics of sildenalfil in children. Therefore, we aimed to carry out a pharmacokinetic study of sildenafil in children with PAH using a single dose. METHODS: Twelve children diagnosed with PAH, consisting of with ten males and two females, were recruited for the study after obtaining written consent from their parents or guardians...
December 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28707968/accelerometry-improving-objective-assessments-of-therapeutic-impact-in-pediatric-pulmonary-arterial-hypertension
#16
Eric D Austin, Jeffrey A Feinstein
No abstract text is available yet for this article.
July 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#17
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28701597/randomized-comparative-study-of-intravenous-infusion-of-three-different-fixed-doses-of-milrinone-in-pediatric-patients-with-pulmonary-hypertension-undergoing-open-heart-surgery
#18
Neeraj Kumar Barnwal, Sanjeeta Rajendra Umbarkar, Manjula Sudeep Sarkar, Raylene J Dias
BACKGROUND: Pulmonary hypertension secondary to congenital heart disease is a common problem in pediatric patients presenting for open heart surgery. Milrinone has been shown to reduce pulmonary vascular resistance and pulmonary artery pressure in pediatric patients and neonates postcardiac surgery. We aimed to evaluate the postoperative outcome in such patients with three different fixed maintenance doses of milrinone. METHODOLOGY: Patients were randomized into three groups...
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28699846/safety-and-effectiveness-of-tadalafil-in-pediatric-patients-with-pulmonary-arterial-hypertension-a-sub-group-analysis-based-on-japan-post-marketing-surveillance
#19
Hiroyoshi Yamazaki, Noriko Kobayashi, Masanori Taketsuna, Koyuki Tajima, Nahoko Suzuki, Masahiro Murakami
OBJECTIVE: To evaluate the long-term safety and effectiveness of tadalafil in pediatric patients with pulmonary arterial hypertension (PAH) in real-world clinical practice. METHODS: This is an observational surveillance of PAH patients receiving tadalafil in the contracted sites. A sub-group analysis was performed of 391 pediatric PAH patients (<18 years) who were included from 1,704 total patients in this surveillance. Safety was assessed from the frequency of adverse drug reactions (ADRs), discontinuations due to adverse events (AEs), and serious adverse drug reactions (SADRs)...
July 20, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28680572/pulmonary-arterial-hypertension-in-the-usa-an-epidemiological-study-in-a-large-insured-pediatric-population
#20
Lin Li, Susan Jick, Stefanie Breitenstein, Gemzel Hernandez, Alexander Michel, David Vizcaya
Pulmonary arterial hypertension (PAH) is rare in children and few data are available in a pediatric general population. This study aims to calculate the annual incidence and prevalence of PAH and to describe these children in a large US population of patients aged under 18 years. Using the US MarketScan claims database we identified 695 children with PAH in 2010-2013. We calculated annual incidence rates and prevalence overall, by age and PAH type (idiopathic and non-idiopathic) using Byar's method. We also described characteristics, co-morbidities, treatment patterns, and diagnostic procedures for these children...
March 2017: Pulmonary Circulation
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