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Pediatric Heart Network

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https://www.readbyqxmd.com/read/29351346/the-congenital-heart-disease-genetic-network-study-cohort-description
#1
Thanh T Hoang, Elizabeth Goldmuntz, Amy E Roberts, Wendy K Chung, Jennie K Kline, John E Deanfield, Alessandro Giardini, Adolfo Aleman, Bruce D Gelb, Meghan Mac Neal, George A Porter, Richard Kim, Martina Brueckner, Richard P Lifton, Sharon Edman, Stacy Woyciechowski, Laura E Mitchell, A J Agopian
The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects) and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot) and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome). Cases with CHDs were recruited through ten sites, 2010-2014...
2018: PloS One
https://www.readbyqxmd.com/read/29340730/age-dependent-association-between-pre-transplant-blood-transfusion-and-outcomes-of-pediatric-heart-transplantation
#2
C McKee, D Tumin, B R Alevriadou, K K Nicol, A R Yates, D Hayes, J D Tobias
Avoidance of red blood cell (RBC) transfusions in patients awaiting heart transplantation (HTx) has been suggested to minimize the risk of allosensitization. Although recent studies have suggested that an immature immune system in younger HTx recipients may reduce risks associated with RBC transfusion, the role of age in moderating the influence of transfusion on HTx outcomes remains unclear. We used available data from a national transplant registry to explore whether the association between pre-transplant transfusions and outcomes of pediatric HTx varies by patient age...
January 16, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29279413/association-between-diastolic-blood-pressure-during-pediatric-in-hospital-cardiopulmonary-resuscitation-and-survival
#3
Robert A Berg, Robert M Sutton, Ron W Reeder, John T Berger, Christopher J Newth, Joseph A Carcillo, Patrick S McQuillen, Kathleen L Meert, Andrew R Yates, Rick E Harrison, Frank W Moler, Murray M Pollack, Todd C Carpenter, David L Wessel, Tammara L Jenkins, Daniel A Notterman, Richard Holubkov, Robert F Tamburro, J Michael Dean, Vinay M Nadkarni
Background -Based on laboratory cardiopulmonary resuscitation (CPR) investigations and limited adult data demonstrating that survival depends on attaining adequate arterial diastolic blood pressure (DBP) during CPR, American Heart Association recommends using BP to guide pediatric CPR. However, evidence-based BP targets during pediatric CPR remain an important knowledge gap for CPR guidelines. Methods -All children ≥37 weeks gestation and <19 years old in Collaborative Pediatric Critical Care Research Network intensive care units with chest compressions for ≥1 minute and invasive arterial blood pressure monitoring prior to and during CPR between July 1, 2013 and June 31, 2016 were included...
December 26, 2017: Circulation
https://www.readbyqxmd.com/read/29258675/the-number-of-refusals-for-donor-organ-quality-does-not-impact-heart-transplant-outcomes-in-children
#4
Raheel Rizwan, Farhan Zafar, Roosevelt Bryant, James S Tweddell, Angela Lorts, Clifford Chin, David L Morales
BACKGROUND: Waitlist mortality is more than 12% for pediatric heart transplantation, with strikingly high rates of organ refusal, many of which are due to donor quality. However, some centers use these organs despite refusals by other centers for donor quality. We hypothesize that the number of refusals for donor quality (RDQ) does not affect pediatric heart transplantation outcomes. METHODS: Pediatric heart transplants from 2000 to 2015 were identified using the United Network for Organ Sharing database and were matched against the potential transplant recipients dataset with donor refusal codes...
December 16, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29226563/fatal-cardiac-arrest-in-pediatric-heart-transplant-recipients-query-of-the-unos-database
#5
Robert W Loar, Susan W Denfield, Shaine A Morris, Hari P Tunuguntla, Antonio G Cabrera, Jack F Price, Wei Zhang, Katherine Hosek, Jeffrey J Kim, William J Dreyer, Aamir Jeewa
The incidence of death by CA after PHTx is unknown. We aimed to determine the incidence and factors for fatal CA after PHTx, and whether a PM affects survival. Retrospective cohort study utilizing the United Network of Organ Sharing registry of patients transplanted ≤18 years. Multivariable analyses in hazard-function domain and Kaplan-Meier analyses were performed for an outcome of death due to CA. There were 7719 PHTx patients queried. CA was the reported cause of death in 11%. Age ≥13 years at time of transplant, presence of a PM, and depressed EF were identified as significant factors for fatal CA...
December 10, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29223416/cost-variation-across-centers-for-the-norwood-operation
#6
Kimberly E McHugh, Sara K Pasquali, Matthew A Hall, Mark A Scheurer
BACKGROUND: The Norwood operation is associated with high health care utilization, and prior studies reported substantial variability in Norwood costs across centers. However, specific factors driving this cost variation are unclear. We assessed center variability in Norwood costs and underlying mechanisms in a multicenter cohort. METHODS: Clinical data from the Pediatric Heart Network Single Ventricle Reconstruction trial were linked with cost data from the Children's Hospital Association Inpatient Essentials database...
December 6, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29170024/exploiting-a-water-network-to-achieve-enthalpy-driven-bromodomain-selective-bet-inhibitors
#7
William R Shadrick, Peter J Slavish, Sergio C Chai, Brett Waddell, Michele Connelly, Jonathan A Low, Cynthia Tallant, Brandon M Young, Nagakumar Bharatham, Stefan Knapp, Vincent A Boyd, Marie Morfouace, Martine F Roussel, Taosheng Chen, Richard E Lee, R Kiplin Guy, Anang A Shelat, Philip M Potter
Within the last decade, the Bromodomain and Extra-Terminal domain family (BET) of proteins have emerged as promising drug targets in diverse clinical indications including oncology, auto-immune disease, heart failure, and male contraception. The BET family consists of four isoforms (BRD2, BRD3, BRD4, and BRDT/BRDT6) which are distinguished by the presence of two tandem bromodomains (BD1 and BD2) that independently recognize acetylated-lysine (KAc) residues and appear to have distinct biological roles. BET BD1 and BD2 bromodomains differ at five positions near the substrate binding pocket: the variation in the ZA channel induces different water networks nearby...
November 4, 2017: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/29155486/toxic-stress-and-child-refugees
#8
John S Murray
The purpose of this article was to describe the phenomenon of toxic stress and its impact on the physical and mental health of child refugees. Almost two decades ago, researchers found that recurring adverse childhood events (ACEs; e.g., physical, psychological, and sexual abuse, neglect, and household dysfunction such as substance abuse, mental illness, and criminal behavior) were associated with a significant increase in serious illnesses during adulthood. Illnesses include heart, lung, and liver disease, cancer, and bone fractures...
November 20, 2017: Journal for Specialists in Pediatric Nursing: JSPN
https://www.readbyqxmd.com/read/29138232/relationship-of-echocardiographic-z-scores-adjusted-for-body-surface-area-to-age-sex-race-and-ethnicity-the-pediatric-heart-network-normal-echocardiogram-database
#9
Leo Lopez, Steven Colan, Mario Stylianou, Suzanne Granger, Felicia Trachtenberg, Peter Frommelt, Gail Pearson, Joseph Camarda, James Cnota, Meryl Cohen, Andreea Dragulescu, Michele Frommelt, Olukayode Garuba, Tiffanie Johnson, Wyman Lai, Joseph Mahgerefteh, Ricardo Pignatelli, Ashwin Prakash, Ritu Sachdeva, Brian Soriano, Jonathan Soslow, Christopher Spurney, Shubhika Srivastava, Carolyn Taylor, Poonam Thankavel, Mary van der Velde, LuAnn Minich
BACKGROUND: Published nomograms of pediatric echocardiographic measurements are limited by insufficient sample size to assess the effects of age, sex, race, and ethnicity. Variable methodologies have resulted in a wide range of Z scores for a single measurement. This multicenter study sought to determine Z scores for common measurements adjusted for body surface area (BSA) and stratified by age, sex, race, and ethnicity. METHODS AND RESULTS: Data collected from healthy nonobese children ≤18 years of age at 19 centers with a normal echocardiogram included age, sex, race, ethnicity, height, weight, echocardiographic images, and measurements performed at the Core Laboratory...
November 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29133477/reproducibility-of-left-ventricular-dimension-versus-area-versus-volume-measurements-in-pediatric-patients-with-dilated-cardiomyopathy
#10
Elif Seda Selamet Tierney, Danielle Hollenbeck-Pringle, Caroline K Lee, Karen Altmann, Carolyn Dunbar-Masterson, Fraser Golding, Minmin Lu, Stephen G Miller, Kimberly Molina, Shobha Natarajan, Carolyn L Taylor, Felicia Trachtenberg, Steven D Colan
BACKGROUND: Multiple echocardiographic methods are used to measure left ventricular size and function. Clinical management is based on individual evaluations and longitudinal trends. The Pediatric Heart Network VVV study (Ventricular Volume Variability) in pediatric patients with dilated cardiomyopathy has reported reproducibility of several of these measures, and how disease state and number of beats impact their reproducibility. In this study, we investigated the impact of observer and sonographer variation on reproducibility of dimension, area, and volume methods to determine the best method for both individual and sequential evaluations...
November 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29073953/temporary-circulatory-support-in-u-s-children-awaiting-heart-transplantation
#11
Vamsi V Yarlagadda, Katsuhide Maeda, Yulin Zhang, Sharon Chen, John C Dykes, Mary Alice Gowen, Paul Shuttleworth, Jenna M Murray, Andrew Y Shin, Olaf Reinhartz, David N Rosenthal, Doff B McElhinney, Christopher S Almond
BACKGROUND: Extracorporeal membrane oxygenation (ECMO) has long served as the standard of care for short-term mechanical circulatory support in pediatrics. It is unknown whether newer-generation temporary circulatory support (TCS) devices afford children a meaningful survival advantage over ECMO. OBJECTIVES: This study sought to determine whether bridge-to-heart transplant survival with a TCS device is superior to ECMO after adjusting for patient differences. METHODS: All children ≤21 years of age listed for heart transplant from 2011 to 2015 who received a TCS device or ECMO as a bridge to transplant were identified using Organ Procurement and Transplantation Network data...
October 31, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28971577/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-transposition-of-the-great-arteries-after-arterial-switch-operation
#12
Carissa M Baker-Smith, Karina Carlson, Jose Ettedgui, Takeshi Tsuda, K Anitha Jayakumar, Matthew Park, Nikola Tede, Karen Uzark, Craig Fleishman, David Connuck, Maggie Likes, Daniel J Penny
OBJECTIVE: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO). DESIGN: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology...
October 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28930815/variability-in-usual-care-mechanical-ventilation-for-pediatric-acute-respiratory-distress-syndrome-time-for-a-decision-support-protocol
#13
Christopher J L Newth, Katherine A Sward, Robinder G Khemani, Kent Page, Kathleen L Meert, Joseph A Carcillo, Thomas P Shanley, Frank W Moler, Murray M Pollack, Heidi J Dalton, David L Wessel, John T Berger, Robert A Berg, Rick E Harrison, Richard Holubkov, Allan Doctor, J Michael Dean, Tammara L Jenkins, Carol E Nicholson
OBJECTIVES: Although pediatric intensivists philosophically embrace lung protective ventilation for acute lung injury and acute respiratory distress syndrome, we hypothesized that ventilator management varies. We assessed ventilator management by evaluating changes to ventilator settings in response to blood gases, pulse oximetry, or end-tidal CO2. We also assessed the potential impact that a pediatric mechanical ventilation protocol adapted from National Heart Lung and Blood Institute acute respiratory distress syndrome network protocols could have on reducing variability by comparing actual changes in ventilator settings to those recommended by the protocol...
November 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28822746/outcomes-of-early-adolescent-donor-hearts-in-adult-transplant-recipients
#14
Shivank Madan, Snehal R Patel, Peter Vlismas, Omar Saeed, Sandhya Murthy, Stephen Forest, William Jakobleff, Daniel Sims, Jacqueline M Lamour, Daphne T Hsu, Julia Shin, Daniel Goldstein, Ulrich P Jorde
OBJECTIVES: This study sought to determine outcomes of adult recipients of early adolescent (EA) (10 to 14 years) donor hearts. BACKGROUND: Despite a shortage of donor organs, EA donor hearts (not used for pediatric patients) are seldom used for adults because of theoretical concerns for lack of hormonal activation and changes in left ventricular mass. Nonetheless, the outcomes of adult transplantation using EA donor hearts are not clearly established. METHODS: All adult (≥18 years of age) heart transplant recipients in the United Network for Organ Sharing database between April 1994 and September 2015 were eligible for this analysis...
August 3, 2017: JACC. Heart Failure
https://www.readbyqxmd.com/read/28734628/adrenergic-receptor-genotypes-influence-postoperative-outcomes-in-infants-in-the-single-ventricle-reconstruction-trial
#15
Ronand Ramroop, George Manase, Danny Lu, Dorin Manase, Shan Chen, Richard Kim, Teresa Lee, William T Mahle, Kimberly McHugh, Mike Mitchell, Martin Tristani-Firouzi, Stephanie B Wechsler, Nicole S Wilder, Victor Zak, Myriam Lafreniere-Roula, Jane W Newburger, J William Gaynor, Mark W Russell, Seema Mital
OBJECTIVES: Adrenergic receptor (ADR) genotypes have been associated with adverse outcomes in heart failure. Our objective was to evaluate the association of ADR genotypes with post-Norwood outcomes in infants with hypoplastic left heart syndrome (HLHS). METHODS: Infants with HLHS participating in the Pediatric Heart Network Single-Ventricle Reconstruction Trial underwent genotyping for 4 single-nucleotide polymorphisms in 3 ADR genes: ADRB1_231A/G, ADRB1_1165G/C, ADRB2_5318C/G, and ADRA2A_2790C/T...
November 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28712678/impact-of-the-18th-birthday-on-waitlist-outcomes-among-young-adults-listed-for-heart-transplant-a-regression-discontinuity-analysis
#16
David M Peng, Qiuying Qu, Nancy McDonald, Seth A Hollander, Daniel Bernstein, Katsuhide Maeda, Beth D Kaufman, David N Rosenthal, Doff B McElhinney, Christopher S Almond
BACKGROUND: Patients listed for heart transplant after their 18th birthday purportedly wait longer to receive a donor heart compared with patients listed before their 18th birthday. It is unclear whether there is an actual difference in wait times and whether any difference in wait time is associated with lower likelihood of transplant and/or higher risk of mortality. METHODS: Organ procurement and transplant network data were used to identify all patients listed for heart transplant between 2006 and 2014 within a 1-year period before and after their 18th birthday...
November 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28711963/transfer-of-neonates-with-critical-congenital-heart-disease-within-a-regionalized-network
#17
Michael F Swartz, Jill M Cholette, Jennifer M Orie, Marshall L Jacobs, Jeffrey P Jacobs, George M Alfieris
Regionalization of pediatric cardiac surgical care varies between and within states. In most geographic regions, at least some neonates with critical heart disease are transferred from their birth hospital to a different hospital for surgery. The impact of neonatal transfer for surgery, particularly over a considerable distance (>10 miles), has been largely unexplored. We sought to examine the impact of transferring neonates for cardiac surgery. We queried the New York State Cardiac Surgery database (2005-2014) from a single institution to identify neonates born within the cardiac surgery center and those transferred for surgery...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28690004/health-insurance-coverage-among-young-adult-survivors-of-pediatric-heart-transplantation
#18
Dmitry Tumin, Susan S Li, Deipanjan Nandi, Robert J Gajarski, Christopher McKee, Joseph D Tobias, Don Hayes
OBJECTIVE: To describe the change in health insurance after heart transplantation among adolescents, and characterize the implications of this change for long-term transplant outcomes. STUDY DESIGN: Patients age 15-18 years receiving first-time heart transplantation between 1999 and 2011 were identified in the United Network for Organ Sharing registry and included in the analysis if they survived at least 5 years. The primary exposure was change or continuity of health insurance coverage between the time of transplant and the 5-year follow-up...
September 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28687711/the-optimal-timing-of-stage-2-palliation-for-hypoplastic-left-heart-syndrome-an-analysis-of-the-pediatric-heart-network-single-ventricle-reconstruction-trial-public-data-set
#19
RANDOMIZED CONTROLLED TRIAL
James M Meza, Edward J Hickey, Eugene H Blackstone, Robert D B Jaquiss, Brett R Anderson, William G Williams, Sally Cai, Glen S Van Arsdell, Tara Karamlou, Brian W McCrindle
BACKGROUND: In infants requiring 3-stage single-ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage 2 palliation (S2P), a physician-modifiable factor, on long-term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics...
October 31, 2017: Circulation
https://www.readbyqxmd.com/read/28625502/a-computable-phenotype-improves-cohort-ascertainment-in-a-pediatric-pulmonary-hypertension-registry
#20
Alon Geva, Jessica L Gronsbell, Tianxi Cai, Tianrun Cai, Shawn N Murphy, Jessica C Lyons, Michelle M Heinz, Marc D Natter, Nandan Patibandla, Jonathan Bickel, Mary P Mullen, Kenneth D Mandl
OBJECTIVES: To compare registry and electronic health record (EHR) data mining approaches for cohort ascertainment in patients with pediatric pulmonary hypertension (PH) in an effort to overcome some of the limitations of registry enrollment alone in identifying patients with particular disease phenotypes. STUDY DESIGN: This study was a single-center retrospective analysis of EHR and registry data at Boston Children's Hospital. The local Informatics for Integrating Biology and the Bedside (i2b2) data warehouse was queried for billing codes, prescriptions, and narrative data related to pediatric PH...
September 2017: Journal of Pediatrics
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