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Critical congenital heart disease

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https://www.readbyqxmd.com/read/28097194/the-mystery-of-the-z-score
#1
REVIEW
Alexander E Curtis, Tanya A Smith, Bulat A Ziganshin, John A Elefteriades
Reliable methods for measuring the thoracic aorta are critical for determining treatment strategies in aneurysmal disease. Z-scores are a pragmatic alternative to raw diameter sizes commonly used in adult medicine. They are particularly valuable in the pediatric population, who undergo rapid changes in physical development. The advantage of the Z-score is its inclusion of body surface area (BSA) in determining whether an aorta is within normal size limits. Therefore, Z-scores allow us to determine whether true pathology exists, which can be challenging in growing children...
August 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/28094679/a-retrospective-analysis-of-a-pediatric-tele-echocardiography-service-to-treat-triage-and-reduce-trans-pacific-transport
#2
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/28092064/critical-congenital-heart-disease-newborn-screening-implementation-lessons-learned
#3
Monica R McClain, John S Hokanson, Regina Grazel, Kim Naarden Van Braun, Lorraine F Garg, Michelle R Morris, Kathleen Moline, Keri Urquhart, Amy Nance, Harper Randall, Marci K Sontag
Introduction The purpose of this article is to present the collective experiences of six federally-funded critical congenital heart disease (CCHD) newborn screening implementation projects to assist federal and state policy makers and public health to implement CCHD screening. Methods A qualitative assessment and summary from six demonstration project grantees and other state representatives involved in the implementation of CCHD screening programs are presented in the following areas: legislation, provider and family education, screening algorithms and interpretation, data collection and quality improvement, telemedicine, home and rural births, and neonatal intensive care unit populations...
January 16, 2017: Maternal and Child Health Journal
https://www.readbyqxmd.com/read/28081558/should-new-zealand-introduce-nationwide-pulse-oximetry-screening-for-the-detection-of-critical-congenital-heart-disease-in-newborn-infants
#4
Elza Cloete, Thomas L Gentles, Jane M Alsweiler, Lesley A Dixon, Dianne R Webster, Deborah L Rowe, Frank H Bloomfield
No abstract text is available yet for this article.
January 13, 2017: New Zealand Medical Journal
https://www.readbyqxmd.com/read/28078382/timing-and-mode-of-delivery-in-prenatally-diagnosed-congenital-heart-disease-an-analysis-of-practices-within-the-university-of-california-fetal-consortium-ucfc
#5
Shabnam Peyvandi, Tina Ahn Thu Thi Nguyen, Myriam Almeida-Jones, Nina Boe, Laila Rhee, Tracy Anton, Mark Sklansky, Maryam Tarsa, Gary Satou, Anita J Moon-Grady
Prenatal diagnosis of critical congenital heart disease (CHD) is associated with decreased morbidity. It is also associated with lower birth weights and earlier gestational age at delivery. The University of California Fetal Consortium (UCfC) comprises five tertiary medical centers, and was created to define treatment practices. We utilized this consortium to assess delivery patterns and outcomes in subjects with prenatal and postnatal diagnosis of CHD. A retrospective cohort study was conducted on maternal-neonatal pairs diagnosed with complex CHD prenatally (n = 186) and postnatally (n = 110) from 2011 to 2013...
January 11, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28075515/the-limitations-of-pulse-oximetry-for-cchd-screening-in-the-nicu
#6
Nithi Fernandes, Satyan Lakshminrusimha
We have read this recently published manuscript by Hu et al(1) regarding using pulse oximetry for screening for critical congenital heart disease (CCHD) in the neonatal intensive care units (NICU) with great interest. As an institution exclusively serving outborn neonates, the study was appropriately designed and executed. Prior published reports of CCHD screening in the NICU report a very low detection rate with pulse oximetry screening. This article is protected by copyright. All rights reserved.
January 11, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28063770/a-post-operative-feeding-protocol-to-improve-outcomes-for-neonates-with-critical-congenital-heart-disease
#7
Jennifer Newcombe, Eileen Fry-Bowers
Neonates with critical congenital heart disease (CCHD) are vulnerable to malnutrition during the post-operative period due to hypermetabolism and hypercatabolism. To improve nutritional outcomes during hospitalization, a nurse led post-operative enteral feeding protocol was implemented at a large U.S. children's hospital. During an eight-month implementation period, twenty-one neonates met protocol inclusion criteria. Days for neonates to achieve goal caloric feedings (120kcal/kg/day) were decreased. A one-way repeated measures analysis of variance showed serum albumin levels and serial anthropometric measurements improved significantly throughout hospitalization (p<0...
January 4, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28043739/canadian-cardiovascular-society-canadian-pediatric-cardiology-association-position-statement-on-pulse-oximetry-screening-in-newborns-to-enhance-detection-of-critical-congenital-heart-disease
#8
Kenny K Wong, Anne Fournier, Deborah S Fruitman, Lisa Graves, Derek G Human, Michael Narvey, Jennifer L Russell
Congenital heart disease is the most common congenital malformation and approximately 3 in 1000 newborns have critical congenital heart disease (CCHD). Timely diagnosis affects morbidity, mortality, and disability, and newborn pulse oximetry screening has been studied to enhance detection of CCHD. In this position statement we present an evaluation of the literature for pulse oximetry screening. Current detection strategies including prenatal ultrasound examination and newborn physical examination are limited by low diagnostic sensitivity...
October 26, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28040762/glial-fibrillary-acidic-protein-plasma-levels-are-correlated-with-degree-of-hypothermia-during-cardiopulmonary-bypass-in-congenital-heart-disease-surgery
#9
Luca Vedovelli, Massimo Padalino, Sara D'Aronco, Giovanni Stellin, Carlo Ori, Virgilio P Carnielli, Manuela Simonato, Paola Cogo
OBJECTIVES: Improved congenital heart defect (CHD) operations have reduced operative mortality to 3%. The major concern is now long-term neurological outcomes. We measured plasma glial fibrillary acidic protein (GFAP), an early marker of brain injury, during different phases of cardiopulmonary bypass (CPB), to correlate the increase of GFAP to clinical parameters or specific operative phases. METHODS: We performed a prospective, single-centre, observational study in children undergoing cardiac operations...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28033081/clinical-databases-and-registries-in-congenital-and-pediatric-cardiac-surgery-cardiology-critical-care-and-anesthesiology-worldwide
#10
David F Vener, Michael Gaies, Jeffrey P Jacobs, Sara K Pasquali
The growth in large-scale data management capabilities and the successful care of patients with congenital heart defects have coincidentally paralleled each other for the last three decades, and participation in multicenter congenital heart disease databases and registries is now a fundamental component of cardiac care. This manuscript attempts for the first time to consolidate in one location all of the relevant databases worldwide, including target populations, specialties, Web sites, and participation information...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28033078/the-accuracy-of-noninvasive-peripheral-pulse-oximetry-after-palliative-cardiac-surgery-in-patients-with-cyanotic-congenital-heart-disease
#11
Gemma E Scrimgeour, Michael J Griksaitis, John V Pappachan, Andrew J Baldock
BACKGROUND: Children with cyanotic congenital heart disease (CCHD) live with oxyhemoglobin saturations that are typically expressed as percentages in the range of 70s and 80s. Peripheral pulse oximetry (measurement of SpO2) performs poorly in this range and yet is widely used to inform clinical decisions in these patients. The reference standard is co-oximetry of arterial samples (SaO2). METHODS: In this study, 515 paired measurements of SpO2 and SaO2 were taken from 19 children who had undergone palliative cardiac surgery...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28011844/the-visible-heart%C3%A2-project-and-free-access-website-atlas-of-human-cardiac-anatomy
#12
Paul A Iaizzo
AIMS: Pre- and post-evaluations of implantable cardiac devices require innovative and critical testing in all phases of the design process. The Visible Heart(®) Project was successfully launched in 1997 and 3 years later the Atlas of Human Cardiac Anatomy website was online. The Visible Heart(®) methodologies and Atlas website can be used to better understand human cardiac anatomy, disease states and/or to improve cardiac device design throughout the development process. METHODS AND RESULTS: To date, Visible(®) Heart methodologies have been used to reanimate 75 human hearts, all considered non-viable for transplantation...
December 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27998861/-risk-factors-for-acute-respiratory-distress-syndrome-following-surgeries-for-pediatric-critical-and-complex-congenital-heart-disease
#13
Shu-Rong Gong, Ying-Rui Zhang, Rong-Guo Yu
OBJECTIVE: To explore the risk factors for acute respiratory distress syndrome (ARDS) in children receiving surgeries for critical and complex congenital heart disease (CCHD). METHODS: According to the 2011's Berlin definition of ARDS, the clinical data were collected from 75 children without ARDS (group I) and 80 children with ARDS (group II) following surgeries for CCHD performed in the Department of Cardiac Surgery of our hospital from January, 2009 to May, 2014...
December 20, 2016: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/27986330/making-the-quick-diagnosis-a-case-of-neonatal-shock
#14
Mike Gardiner, Timothy K Ruttan, Andrew J Kienstra, Matthew Wilkinson
BACKGROUND: The work-up and initial management of a critically ill neonate is challenging and anxiety provoking for the Emergency Physician. While sepsis and critical congenital heart disease represent a large proportion of neonates presenting to the Emergency Department (ED) in shock, there are several additional etiologies to consider. Underlying metabolic, endocrinologic, gastrointestinal, neurologic, and traumatic disorders must be considered in a critically ill infant. Several potential etiologies will present with nonspecific and overlapping signs and symptoms, and the diagnosis often is not evident at the time of ED assessment...
December 13, 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27959615/developmental-mechanisms-of-aortic-valve-malformation-and-disease
#15
Bingruo Wu, Yidong Wang, Feng Xiao, Jonathan T Butcher, Katherine E Yutzey, Bin Zhou
Normal aortic valves are composed of valve endothelial cells (VECs) and valve interstitial cells (VICs). VICs are the major cell population and have distinct embryonic origins in the endocardium and cardiac neural crest cells. Cell signaling between the VECs and VICs plays critical roles in aortic valve morphogenesis. Disruption of major cell signaling pathways results in aortic valve malformations, including bicuspid aortic valve (BAV). BAV is a common congenital heart valve disease that may lead to calcific aortic valve disease (CAVD), but there is currently no effective medical treatment for this beyond surgical replacement...
December 9, 2016: Annual Review of Physiology
https://www.readbyqxmd.com/read/27928258/evaluation-of-pulse-oximetry-in-the-early-detection-of-cyanotic-congenital-heart-disease-in-newborns
#16
Amir Hosein Movahedian, Ziba Mosayebi, Setareh Sagheb
Background: Delayed or missed diagnosis of critical and cyanotic congenital heart disease (CHD) in asymptomatic newborns may result in significant morbidity and mortality. The aim of this study was to determine the accuracy of pulse oximetry screening performed on the first day of life for the early detection of critical and cyanotic CHD in apparently normal newborns. Methods: This cross-sectional study used postductal pulse oximetry to evaluate term neonates born between 2008 and 2011 with normal physical examinations...
April 13, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/27911332/type-0-spinal-muscular-atrophy-further%C3%A2-delineation-of-prenatal-and%C3%A2-postnatal-features-in-16-patients
#17
Sarah Grotto, Jean-Marie Cuisset, Stéphane Marret, Séverine Drunat, Patricia Faure, Séverine Audebert-Bellanger, Isabelle Desguerre, Vincent Flurin, Anne-Gaëlle Grebille, Anne-Marie Guerrot, Hubert Journel, Gilles Morin, Ghislaine Plessis, Sylvain Renolleau, Joëlle Roume, Brigitte Simon-Bouy, Renaud Touraine, Marjolaine Willems, Thierry Frébourg, Eric Verspyck, Pascale Saugier-Veber
BACKGROUND: Spinal muscular atrophy (SMA) is caused by homozygous inactivation of the SMN1 gene. The SMN2 copy number modulates the severity of SMA. The 0SMN1/1SMN2 genotype, the most severe genotype compatible with life, is expected to be associated with the most severe form of the disease, called type 0 SMA, defined by prenatal onset. OBJECTIVE: The aim of the study was to review clinical features and prenatal manifestations in this rare SMA subtype. METHODS: SMA patients with the 0SMN1/1SMN2 genotype were retrospectively collected using the UMD-SMN1 France database...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27901294/performance-of-functional-fibrinogen-thromboelastography-in-children-undergoing-congenital-heart-surgery
#18
Nischal K Gautam, Chunyan Cai, Olga Pawelek, Muhammad B Rafique, Davide Cattano, Evan G Pivalizza
BACKGROUND: Functional Fibrinogen assay of the Thromboelastography (FFTEG), a whole blood viscoelastic hemostatic assay, has been used to estimate fibrinogen levels in adult patients undergoing major surgery but its performance in pediatric patients undergoing cardiac surgery requires evaluation. In this study, we evaluate the correlation between FFTEG parameters and standard laboratory tests for fibrinogen and platelet counts before and after cardiopulmonary bypass in children undergoing repair for congenital heart disease...
February 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27899284/defective-lymphatic-valve-development-and-chylothorax-in-mice-with-a-lymphatic-specific-deletion-of-connexin43
#19
Stephanie J Munger, Michael J Davis, Alexander M Simon
Lymphatic valves (LVs) are cusped luminal structures that permit the movement of lymph in only one direction and are therefore critical for proper lymphatic vessel function. Congenital valve aplasia or agenesis can, in some cases, be a direct cause of lymphatic disease. Knowledge about the molecular mechanisms operating during the development and maintenance of LVs may thus aid in the establishment of novel therapeutic approaches to treat lymphatic disorders. In this study, we examined the role of Connexin43 (Cx43), a gap junction protein expressed in lymphatic endothelial cells (LECs), during valve development...
January 15, 2017: Developmental Biology
https://www.readbyqxmd.com/read/27849656/the-use-of-nesiritide-in-children-with-congenital-heart-disease
#20
Ronald A Bronicki, Michele Domico, Paul A Checchia, Curtis E Kennedy, Ayse Akcan-Arikan
OBJECTIVE: We evaluated the use of nesiritide in children with critical congenital heart disease, pulmonary congestion, and inadequate urine output despite conventional diuretic therapy. DESIGN: We conducted a retrospective analysis of 26 consecutive patients, comprising 37 infusions occurring during separate hospitalizations. Hemodynamic variables, urine output, and serum creatinine levels were monitored prior to and throughout the duration of therapy with nesiritide...
November 15, 2016: Pediatric Critical Care Medicine
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