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Pediatric Arterial hypertension

Akimichi Shibata, Hiroki Mori, Kazuki Kodo, Toshio Nakanishi, Hiroyuki Yamagishi
BACKGROUND: Recent progress in surgical and intensive care has improved the prognosis of congenital heart disease (CHD) associated with heterotaxy syndrome. Less is known, however, about pulmonary vascular complications in these patients. Methods and Results: We reviewed medical records of 236 patients who were diagnosed with polysplenia syndrome at 2 institutions for pediatric cardiology in Japan from 1978 to 2015. We selected and compared the clinical records of 16 patients with polysplenia who had incomplete atrioventricular septal defect (AVSD) as the polysplenia group, and 22 age-matched patients with incomplete AVSD without any syndromes including polysplenia as the control group...
March 5, 2019: Circulation Journal: Official Journal of the Japanese Circulation Society
Michal Schäfer, D Dunbar Ivy, Steven H Abman, Kurt R Stenmark, Lorna Browne, Alex J Barker, Max B Mitchell, Gareth J Morgan, Neil Wilson, Anar Shah, Madhukar Kollengode, Nivedita Naresh, Brian Fonseca, Michael DiMaria, J Kern Buckner, Kendall S Hunter, Vitaly Oleg Kheyfets, Brett E Fenster, Uyen Truong
Despite different developmental and pathologic processes affecting lung vascular remodeling in both patient populations, differences in 4D-MRI findings between children and adults with PAH have not been studied. The purpose of this study was to compare flow hemodynamic state, including flow-mediated shear forces, between pediatric and adult patients with PAH matched by severity of pulmonary vascular resistance index (PVRi). Adults (n=10) and children (n=10) with PAH matched by pulmonary vascular resistance index (PVRi), and healthy adult (n=10) and pediatric (n=10) subjects underwent comprehensive 4D-Flow MRI to assess peak systolic wall shear stress (WSSmax ) measured in the main, right, and left pulmonary arteries (MPA, RPA, and LPA), viscous energy loss ( EL ) along the MPA-RPA and MPA-LPA tract, and qualitative analysis of secondary flow hemodynamics...
March 1, 2019: American Journal of Physiology. Heart and Circulatory Physiology
Sarah D de Ferranti, Julia Steinberger, Rebecca Ameduri, Annette Baker, Holly Gooding, Aaron S Kelly, Michele Mietus-Snyder, Mark M Mitsnefes, Amy L Peterson, Julie St-Pierre, Elaine M Urbina, Justin P Zachariah, Ali N Zaidi
This scientific statement presents considerations for clinical management regarding the assessment and risk reduction of select pediatric populations at high risk for premature cardiovascular disease, including acquired arteriosclerosis or atherosclerosis. For each topic, the evidence for accelerated acquired coronary artery disease and stroke in childhood and adolescence and the evidence for benefit of interventions in youth will be reviewed. Children and adolescents may be at higher risk for cardiovascular disease because of significant atherosclerotic or arteriosclerotic risk factors, high-risk conditions that promote atherosclerosis, or coronary artery or other cardiac or vascular abnormalities that make the individual more vulnerable to the adverse effects of traditional cardiovascular risk factors...
February 25, 2019: Circulation
Jhao-Jhuang Ding, Shih-Hua Lin, Jin-Yao Lai, Tai-Wei Wu, Jing-Long Huang, Hung-Tao Chung, Min-Hua Tseng
BACKGROUND: Renal artery stenosis is one of the secondary causes of pediatric hypertension. Cases with critical unilateral renal artery stenosis manifesting with the hyponatremic hypertensive syndrome are rare and a comprehensive description of this disorder in the pediatric population is lacking in the literature. CASE PRESENTATION: We describe a 4-year-old boy who presented with severe hypertension, profound hyponatremia, hypokalemia, nephrotic range proteinuria, and polyuria...
February 21, 2019: BMC Nephrology
Shi-Bing Xi, Shu-Shui Wang, Ming-Yang Qian, Yu-Mei Xie, Jun-Jie Li, Zhi-Wei Zhang
BACKGROUND: Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction. METHODS: The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively...
February 15, 2019: Chinese Medical Journal
Heather Cleveland, Sheena Pimpalwar, Daniel Ashton, Alex Chau, Aaditya Nagaraj, J Alberto Hernandez
PURPOSE: To evaluate technical and clinical success and report long-term outcomes of portal vein (PV) recanalization in pediatric orthotopic liver transplant (OLT) patients with chronic PV occlusion. MATERIALS AND METHODS: This is a retrospective review of 15 OLT patients (5 males) with chronic PV occlusion who underwent PV recanalization (33 procedures) between October 2011 and February 2018. Median age was 4.5 years (range, 1-16 years); median weight was 16.6 kg (range, 11...
February 14, 2019: Journal of Vascular and Interventional Radiology: JVIR
Pei-Ni Jone, Dunbar D Ivy
PURPOSE OF REVIEW: The purpose of this review is to detail the advanced echocardiographic studies that evaluated outcomes in pediatric PH using these advanced imaging techniques: (1) right heart size and function (using 2D and 3D echocardiography), (2) myocardial mechanics, and (3) right ventricular (RV) to pulmonary arterial coupling. RECENT FINDINGS: Advanced echocardiographic imaging tools in pediatric PH include RV/left ventricular ratio, 3D echocardiographic assessment of RV volumes and ejection fraction, right atrial and RV strain, and RV-pulmonary arterial coupling ratio that allow for evaluation of clinical outcomes in this patient population...
February 9, 2019: Current Treatment Options in Cardiovascular Medicine
Wenxin Ning, Stephanie Chan, Andrew Beam, Ming Yu, Alon Geva, Katherine Liao, Mary Mullen, Kenneth D Mandl, Isaac Kohane, Tianxi Cai, Sheng Yu
OBJECTIVE: Phenotyping algorithms can efficiently and accurately identify patients with a specific disease phenotype and construct electronic health records (EHR)-based cohorts for subsequent clinical or genomic studies. Previous studies have introduced unsupervised EHR-based feature selection methods that yielded algorithms with high accuracy. However, those selection methods still require expert intervention to tweak the parameter settings according to the EHR data distribution for each phenotype...
February 7, 2019: Journal of Biomedical Informatics
Ingo Franke, Malik Aydin, Lisa Kurylowicz, Corinna Elke Llamas Lopez, Rainer Ganschow, Michael J Lentze, Mark Born
BACKGROUND: Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. average length of stay, complications) as well as of secondary nephrotic syndrome (SNS) is not well known. METHODS: A nationwide ESPED follow-up study presenting the clinical course and management of children with NS in Germany. RESULTS: In course of 2 years, 347 children developed the first onset of NS, hereof 326 patients (93...
February 7, 2019: BMC Nephrology
Ronald A Ligon, Christopher J Petit
We describe a patient with chronic thromboembolic pulmonary hypertension who presented to the pediatric cardiac catheterization laboratory for peripheral pulmonary artery recanalization and angioplasty. This case report outlines serial pulmonary arterial rehabilitation via a retrograde approach through intrapulmonary collateral arterial connections.
February 5, 2019: Catheterization and Cardiovascular Interventions
Laila S Al Yazidi, Julie Huynh, Philip N Britton, C Orla Morrissey, Tony Lai, Glen P Westall, Hiran Selvadurai, Alison Kesson
We present a case of endobronchial fusariosis following bilateral sequential lung transplantation for idiopathic pulmonary arterial hypertension in a 13 years old boy who was treated successfully with posaconazole and nebulized amphotericin B. We discuss the role of nebulized amphotericin B in treating invasive pulmonary fungal disease in children. To our knowledge, this is the first pediatric case of endobronchial fusariosis reported in the literature.
March 2019: Medical Mycology Case Reports
Vitaly Kheyfets, Uyen Truong, Dunbar Ivy, Robin Shandas
Pulmonary hypertension (PH) is a degenerative disease characterized by progressively increased right ventricular (RV) afterload that leads to ultimate functional decline [1]. Recent observational studies have documented a decrease in left ventricular (LV) torsion during ejection, with preserved LV ejection fraction (EF) in pediatric and adult PH patients [2-4]. The objective of this study was to develop a computational model of the bi-ventricular heart and use it to evaluate changes in LV torsion mechanics in response to mechanical, structural, and hemodynamic changes in the RV free-wall...
February 4, 2019: Journal of Biomechanical Engineering
Nesrine Farhat, Frederic Lador, Maurice Beghetti
Paediatric pulmonary arterial hypertension (PAH) remains a rare and severe disease with a poor prognosis. PAH may be idiopathic, heritable or associated to systemic conditions in particular associated with congenital heart disease. Areas covered: A thorough and extensive diagnostic approach is required for a correct diagnosis. Outcome has improved over the last decade with a better diagnostic approach and with the initiation of new targeted therapies. However, there is still significant progress to achieve as there is still no cure for this devastating disease...
January 30, 2019: Expert Review of Cardiovascular Therapy
Luyun Fan, Huimin Zhang, Jun Cai, Lirui Yang, Bin Liu, Dongmei Wei, Jiachen Yu, Jiali Fan, Lei Song, Wenjun Ma, Xianliang Zhou, Haiying Wu, Ying Lou
BACKGROUND: Childhood Takayasu's arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality. This study aims to investigate the clinical course of c-TA and prognostic factors associated with rehospitalization and events including vascular complications, flares, and death. METHODS: An ambispective study of 101 c-TA patients satisfying the American College of Rheumatology (ACR) criteria and/or the European League against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS) criteria was conducted from January 2002 to December 2017...
January 22, 2019: Arthritis Research & Therapy
Arianna Amália Dégi, Adrienn Bárczi, Dolóresz Szabó, Éva Kis, György S Reusz, Antal Dezsőfi
OBJECTIVES: Cardiovascular (CV) diseases play a leading role in the mortality of adult liver transplant (LT) recipients. However, data regarding CV risk factors in children after LT remain sparse. The present study assessed the presence of CV risk factors and signs of CV impairment in LT children. METHODS: A total of 42 LT recipients (21 men, age 9.93 ± 3.57 years) were studied. Body composition [body mass index standard deviation score, percentage of body fat (by bioimpedance analysis)], lipid profiles, glycemic control, blood pressure, and arterial stiffness [assessed by aortic pulse wave velocity (PWV)] were evaluated...
March 2019: Journal of Pediatric Gastroenterology and Nutrition
Ozden Ozgur Horoz, Dincer Yildizdas, Yusuf Sari, Ilker Unal, Faruk Ekinci, Ercument Petmezci
PURPOSE: To the best of our knowledge, in the literature, there is no data regarding clinical utility of the abdominal perfusion pressure (APP) in critically ill children. Thus, in the present study, we aimed to investigate the clinical utility of APP in predicting of survival in critically ill children with IAH. DESIGN: A prospective cohort study of patients between 1 month to 18 years who had risk for intra-abdominal hypertension from June 2013 to January 2014...
December 13, 2018: Journal of Pediatric Surgery
Weiguang Yang, Melody Dong, Marlene Rabinovitch, Frandics P Chan, Alison L Marsden, Jeffrey A Feinstein
Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling resulting in right ventricular (RV) dysfunction and ultimately RV failure. Mechanical stimuli acting on the vessel walls of the full pulmonary tree have not previously been comprehensively characterized. The goal of this study is to characterize wall shear stress (WSS) and strain in pediatric PAH patients at different stages of disease severity using computational patient-specific modeling. Computed tomography, magnetic resonance imaging and right heart catheterization data were collected and assimilated into pulmonary artery (PA) models for patients with and without PAH...
January 12, 2019: Biomechanics and Modeling in Mechanobiology
Pei-Ni Jone, Michal Schäfer, Zhaoxing Pan, D Dunbar Ivy
BACKGROUND: Right ventricular (RV) function is an important determinant of outcomes in pulmonary hypertension (PH). RV-arterial coupling ratio using stroke volume (SV) to end-systolic volume (ESV) has been shown to be an independent predictor of outcome in adults with PH. SV/ESV has not been used in pediatrics to predict outcomes. We compared SV/ESV between pediatric patients with PH, controls, and among groups based on disease severity. We correlated SV/ESV to RV strain and evaluated SV/ESV as a predictor of outcomes in pediatric PH...
December 2019: Circulation. Cardiovascular Imaging
Ashley A Colletti, Taniga Kiatchai, Vivian H Lyons, Bala G Nair, Rosemary M Grant, Monica S Vavilala
BACKGROUND: Traumatic brain injury anesthesia care is complex. The use of clinical decision support to improve pediatric trauma care has not been examined. AIM: To examine feasibility, reliability, and key performance indicators for traumatic brain injury anesthesia care using clinical decision support. METHODS: Clinical decision support was activated for patients under 19 years undergoing craniotomy for suspected traumatic brain injury. Anesthesia providers were prompted to adhere to process measures via on-screen alerts and notified in real time of abnormal monitor data or laboratory results (unwanted key performance indicator events)...
January 4, 2019: Paediatric Anaesthesia
Olaya Hernández-Frías, Helena Gil-Peña, José M Pérez-Roldán, Susana González-Sanchez, Gema Ariceta, Sara Chocrón, Reyner Loza, Francisco de la Cerda Ojeda, Leire Madariaga, Inés Vergara, Marta Fernández-Fernández, Susana Ferrando-Monleón, Montserrat Antón-Gamero, Ángeles Fernández-Maseda, M Isabel Luis-Yanes, Fernando Santos
OBJECTIVE: To find out if cardiovascular alterations are present in pediatric patients with X-linked hypophosphatemia (XLH). STUDY DESIGN: Multicentre prospective clinical study on pediatric patients included in the RenalTube database ( ) with genetically confirmed diagnosis of XLH by mutations in the PHEX gene. The study's protocol consisted of biochemical work-up, 24-h ambulatory blood pressure monitoring (ABPM), carotid ultrasonography, and echocardiogram...
January 4, 2019: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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