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Critical congenital heart disease

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https://www.readbyqxmd.com/read/29345005/-there-s-no-acknowledgement-of-what-this-does-to-people-a-qualitative-exploration-of-mental-health-among-parents-of-children-with-critical-congenital-heart-defects
#1
Sarah Woolf-King, Emily Arnold, Sandra Weiss, David Teitel
AIMS AND OBJECTIVES: The purpose of this study was to explore the psychological impact of parenting a child with a critical congenital heart defect (CHD), and the feasibility and acceptability of integrating psychological services into pediatric cardiology care. BACKGROUND: Children with critical CHD are at an increased risk for long-term behavioral, social, and emotional difficulties. Data suggest that this risk is partially attributable to parental mental health, which is a stronger predictor of long-term behavioral problems in CHD children than disease-specific and surgical factors...
January 18, 2018: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/29310808/frequency-and-consequences-of-influenza-vaccination-in-adults-with-congenital-heart-disease
#2
Idris Bare, Jennifer Crawford, Kendell Pon, Negeen Farida, Payam Dehghani
Immunization against influenza is a critical, but perhaps underappreciated prevention of morbidity and mortality in the cardiac population. The purpose of the present study is to examine influenza vaccination rates in adults with congenital heart disease (ACHD). A secondary purpose is to explore whether there is an association between demographic, medical, and behavioral variables and receipt of the influenza vaccination. Of the 183 consecutive ACHD patients who were contacted, 123 responded to our telephone survey...
December 11, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29290587/rapgef5-regulates-nuclear-translocation-of-%C3%AE-catenin
#3
John N Griffin, Florencia Del Viso, Anna R Duncan, Andrew Robson, Woong Hwang, Saurabh Kulkarni, Karen J Liu, Mustafa K Khokha
Canonical Wnt signaling coordinates many critical aspects of embryonic development, while dysregulated Wnt signaling contributes to common diseases, including congenital malformations and cancer. The nuclear localization of β-catenin is the defining step in pathway activation. However, despite intensive investigation, the mechanisms regulating β-catenin nuclear transport remain undefined. In a patient with congenital heart disease and heterotaxy, a disorder of left-right patterning, we previously identified the guanine nucleotide exchange factor, RAPGEF5...
December 27, 2017: Developmental Cell
https://www.readbyqxmd.com/read/29287130/a-brief-history-of-fetal-echocardiography-and-its-impact-on-the-management-of-congenital-heart-disease
#4
Dev Maulik, Navin C Nanda, Devika Maulik, Gustavo Vilchez
Congenital heart disease (CHD), the most common congenital malformation, is associated with adverse outcome. Development of fetal echocardiography has made prenatal diagnosis of CHD a reality, and in the process revolutionized its management. This historical review briefly narrates this development over the decades focusing on the emergence of the primary modalities of fetal echocardiography comprised of the time-motion mode, two-dimensional B-mode, spectral Doppler, color Doppler, and three- and four-dimensional cardiac imaging...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29286270/landmark-lecture-on-cardiac-intensive-care-and-anaesthesia-continuum-and-conundrums
#5
Peter C Laussen
Cardiac anesthesia and critical care provide an important continuum of care for patients with congenital heart disease. Clinicians in both areas work in complex environments in which the interactions between humans and technology is critical. Understanding our contributions to outcomes (modifiable risk) and our ability to perceive and predict an evolving clinical state (low failure-to-predict rate) are important performance metrics. Improved methods for capturing continuous physiologic signals will allow for new and interactive approaches to data visualization, and for sophisticated and iterative data modeling that will help define a patient's phenotype and response to treatment (precision physiology)...
December 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29282804/in-vitro-modeling-of-congenital-heart-defects-associated-with-an-nkx2-5-mutation-revealed-a-dysregulation-in-bmp-notch-mediated-signaling
#6
Abeer F Zakariyah, Rashida F Rajgara, Ellias Horner, Marie-Elodie Cattin, Alexandre Blais, Ilona S Skerjanc, Patrick G Burgon
The Nkx2-5 gene codes for a transcription factor that plays a critical role in heart development. Heterozygous mutations in NKX2-5 in both human and mice result in congenital heart defects (CHDs). However, the molecular mechanisms by which these mutations cause the disease are still unknown. Recently, we have generated the heterozygous mouse model of the human CHDs associated mutation NKX2-5 R142C (Nkx2-5R141C/+ mouse ortholog of human NKX2-5 R142C variant) that developed septal and conduction defects. This study generated a heterozygous Nkx2-5 R141C mouse embryonic stem cell line (Nkx2-5R141C/+ mESCs) to model CHDs in vitro...
December 28, 2017: Stem Cells
https://www.readbyqxmd.com/read/29279413/association-between-diastolic-blood-pressure-during-pediatric-in-hospital-cardiopulmonary-resuscitation-and-survival
#7
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/29273653/perfusion-index-in-healthy-newborns-during-critical-congenital-heart-disease-screening-at-24-hours-retrospective-observational-study-from-the-usa
#8
Priya Jegatheesan, Matthew Nudelman, Keshav Goel, Dongli Song, Balaji Govindaswami
OBJECTIVE: To describe the distribution of perfusion index (PI) in asymptomatic newborns at 24 hours of life when screening for critical congenital heart disease (CCHD) using an automated data selection method. DESIGN: This is a retrospective observational study. SETTING: Newborn nursery in a California public hospital with ~3500 deliveries annually. METHODS: We developed an automated programme to select the PI values from CCHD screens...
December 21, 2017: BMJ Open
https://www.readbyqxmd.com/read/29250516/use-of-therapeutic-plasma-exchange-during-extracorporeal-life-support-in-critically-ill-cardiac-children-with-thrombocytopenia-associated-multi-organ-failure
#9
Mei Chong, Alejandro J Lopez-Magallon, Lucas Saenz, Mahesh S Sharma, Andrew D Althouse, Victor O Morell, Ricardo Munoz
Background: Thrombocytopenia-associated multi-organ failure (TAMOF) in children is a well-described factor for increased hospital mortality. Low cardiac output syndrome (LCOS) and the effects of cardiopulmonary bypass may manifest with several adverse physiologic and immunologic effects, with varying degrees of thrombocytopenia and multi-organ dysfunction, sometimes very similar to TAMOF. LCOS is a common occurrence in children with critical heart disease, presenting in as much as 23...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29248924/is-there-more-to-zika-complex-cardiac-disease-in-a-case-of-congenital-zika-syndrome
#10
Asimenia Angelidou, Zoe Michael, Arda Hotz, Kevin Friedman, Sitaram Emani, Kerri LaRovere, Helen Christou
The epidemic of Zika virus (ZIKV) has resulted in a surge of newborns with microcephaly and brain abnormalities. In this report, we describe the first case, to our knowledge, of congenital Zika syndrome with concomitant critical congenital heart disease. The mother had a confirmed ZIKV infection in the first trimester of pregnancy. Fetal ultrasonography at 31 weeks of gestation revealed cerebral cortical calcifications and hypoplastic left heart syndrome. The severity of brain involvement was assessed by postnatal magnetic resonance imaging and echocardiogram, and palliative surgery was performed...
December 16, 2017: Neonatology
https://www.readbyqxmd.com/read/29241483/national-trends-and-outcomes-in-isolated-tricuspid-valve-surgery
#11
Chad J Zack, Erin A Fender, Pranav Chandrashekar, Yogesh N V Reddy, Courtney E Bennett, John M Stulak, Virginia M Miller, Rick A Nishimura
BACKGROUND: Severe isolated disease of the tricuspid valve (TV) is increasing and results in intractable right heart failure. However, isolated TV surgery is rarely performed, and there are little data describing surgical outcomes. OBJECTIVES: The purpose of this study was to evaluate contemporary utilization trends and in-hospital outcomes for isolated TV surgery in the United States. METHODS: Patients age >18 years who underwent TV repair or replacement from 2004 to 2013 were identified using the National Inpatient Sample...
December 19, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29209720/association-of-us-state-implementation-of-newborn-screening-policies-for-critical-congenital-heart-disease-with-early-infant-cardiac-deaths
#12
Rahi Abouk, Scott D Grosse, Elizabeth C Ailes, Matthew E Oster
Importance: In 2011, critical congenital heart disease was added to the US Recommended Uniform Screening Panel for newborns, but whether state implementation of screening policies has been associated with infant death rates is unknown. Objective: To assess whether there was an association between implementation of state newborn screening policies for critical congenital heart disease and infant death rates. Design, Setting, and Participants: Observational study with group-level analyses...
December 5, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29209703/the-success-of-state-newborn-screening-policies-for-critical-congenital-heart-disease
#13
EDITORIAL
Alex R Kemper, Wendy K K Lam, Joseph A Bocchini
No abstract text is available yet for this article.
December 5, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29206731/enteral-feeding-practices-in-infants-with-congenital-heart-disease-across-european-picus-a-european-society-of-pediatric-and-neonatal-intensive-care-survey
#14
Lyvonne N Tume, Reinis Balmaks, Eduardo da Cruz, Lynne Latten, Sascha Verbruggen, Frédéric V Valla
OBJECTIVES: To describe enteral feeding practices in pre and postoperative infants with congenital heart disease in European PICUs. DESIGN: Cross-sectional electronic survey. SETTING: European PICUs that admit infants with congenital heart disease pre- and postoperatively. PARTICIPANTS: One senior PICU physician or designated person per unit. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Fifty-nine PICUs from 18 European countries responded to the survey...
December 4, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29206729/extracorporeal-cardiopulmonary-resuscitation-in-the-pediatric-cardiac-population-in-search-of-a-standard-of-care
#15
Javier J Lasa, Parag Jain, Tia T Raymond, Charles G Minard, Alexis Topjian, Vinay Nadkarni, Michael Gaies, Melania Bembea, Paul A Checchia, Lara S Shekerdemian, Ravi Thiagarajan
OBJECTIVES: Although clinical and pharmacologic guidelines exist for the practice of cardiopulmonary resuscitation in children (Pediatric Advanced Life Support), the practice of extracorporeal cardiopulmonary resuscitation in pediatric cardiac patients remains without universally accepted standards. We aim to explore variation in extracorporeal cardiopulmonary resuscitation procedures by surveying clinicians who care for this high-risk patient population. DESIGN: A 28-item cross-sectional survey was distributed via a web-based platform to clinicians focusing on cardiopulmonary resuscitation practices and extracorporeal membrane oxygenation team dynamics immediately prior to extracorporeal membrane oxygenation cannulation...
December 4, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29162633/use-of-prostaglandins-in-duct-dependent-congenital-heart-conditions
#16
Yogen Singh, Paraskevi Mikrou
Congenital heart disease (CHD) remains a leading cause of infant mortality, which is even higher in infants with undiagnosed duct-dependent CHDs. Up to 39%-50% of infants with critical CHD are being discharged undiagnosed from the hospital. Infants with duct-dependent critical CHD remain well during the fetal period and may deteriorate when the ductus arteriosus (commonly called 'duct') closes after birth. It is critical to open or maintain ductus arteriosus patent in infants with duct-dependent CHDs. Prostaglandin E1 (alprostadil marketed as 'Prostin VR ') and prostaglandin E2 (dinoprostone) are used to maintain a patent ductus arteriosus and the dose of medication depends on the clinical presentation...
November 21, 2017: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/29152920/using-state-birth-defects-registries-to-evaluate-regional-critical-congenital-heart-disease-newborn-screening
#17
Amy Peterson Case, Stephanie D Miller, Monica R McClain
BACKGROUND: Most states have now passed legislation mandating pulse oximetry for all newborns, or have promulgated regulations or guidelines to encourage use of routine pulse oximetry. State-based birth defects registries may be well positioned to track and evaluate critical congenital heart disease (CCHD) screening coverage and outcomes. This purpose of this study was to determine: (1) the proportion of cases detected by screening, (2) health services use by children with CCHDs during the first year of life, and (3) mortality outcomes...
November 1, 2017: Birth defects research
https://www.readbyqxmd.com/read/29152906/short-term-outcomes-following-implementation-of-a-dedicated-young-adult-congenital-heart-disease-transition-program
#18
Sumeet S Vaikunth, Roberta G Williams, Merujan Y Uzunyan, Han Tun, Cheryl Barton, Philip M Chang
OBJECTIVE: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease...
November 20, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29145300/cost-effectiveness-analysis-of-neonatal-screening-of-critical-congenital-heart-defects-in-china
#19
Ruoyan Gai Tobe, Gerard R Martin, Fuhai Li, Akinori Moriichi, Bin Wu, Rintaro Mori
BACKGROUND: Pulse oximetry screening is a highly accurate tool for the early detection of critical congenital heart disease (CCHD) in newborn infants. As the technique is simple, noninvasive, and inexpensive, it has potentially significant benefits for developing countries. The aim of this study is to provide information for future clinical and health policy decisions by assessing the cost-effectiveness of CCHD screening in China. METHODS AND FINDINGS: We developed a cohort model to evaluate the cost-effectiveness of screening all Chinese newborns annually using 3 possible screening options compared to no intervention: pulse oximetry alone, clinical assessment alone, and pulse oximetry as an adjunct to clinical assessment...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29133208/homozygous-loss-of-function-brca1-variant-causing-a-fanconi-anemia-like-phenotype-a-clinical-report-and-review-of-previous-patients
#20
Bruna L Freire, Thais K Homma, Mariana F A Funari, Antônio M Lerario, Aline M Leal, Elvira D R P Velloso, Alexsandra C Malaquias, Alexander A L Jorge
BACKGROUND: Fanconi Anemia (FA) is a rare and heterogeneous genetic syndrome. It is associated with short stature, bone marrow failure, high predisposition to cancer, microcephaly and congenital malformation. Many genes have been associated with FA. Previously, two adult patients with biallelic pathogenic variant in Breast Cancer 1 gene (BRCA1) had been identified in Fanconi Anemia-like condition. CLINICAL REPORT: The proband was a 2.5 year-old girl with severe short stature, microcephaly, neurodevelopmental delay, congenital heart disease and dysmorphic features...
November 10, 2017: European Journal of Medical Genetics
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