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Critical congenital heart disease

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https://www.readbyqxmd.com/read/28636109/xq26-1-26-3-duplication-including-mospd1-and-gpc3-identified-in-boy-with-short-stature-and-double-outlet-right-ventricle
#1
Yukiko Hirota, Takaomi Minami, Tomoyuki Sato, Akiko Yokomizo, Auimi Matsumoto, Masahide Goto, Eriko Jinbo, Takanori Yamamgata
Xq25q26 duplication syndrome has been reported in individuals with clinical features such as short stature, intellectual disability, syndromic facial appearance, small hands and feet, and genital abnormalities. The symptoms are related to critical chromosome regions including Xq26.1-26.3. In this particular syndrome, no patient with congenital heart disease was previously reported. Here, we report a 6-year-old boy with typical symptoms of Xq25q26 duplication syndrome and double outlet right ventricle (DORV) with pulmonary atresia (PA)...
June 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28629487/learning-and-memory-in-adolescents-with-critical-biventricular-congenital-heart-disease
#2
Adam R Cassidy, Jane W Newburger, David C Bellinger
OBJECTIVES: Although evidence exists of broadly defined memory impairment among adolescents with critical congenital heart disease (CHD), nuanced investigations of declarative memory in this at-risk population have not been conducted. This study had two primary aims: (1) to conduct a fine-grained analysis of a range of relevant learning and memory processes in adolescents with critical biventricular CHD, and (2) to identify risk, odds, and predictors of memory impairment. METHODS: Data were combined from two single-center studies of neurodevelopmental outcomes in critical CHD...
June 20, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28624838/dental-management-of-pediatric-patients-affected-by-pulmonary-atresia-with-ventricular-septal-defect-a-scoping-review
#3
A Garrocho-Rangel, A-C Echavarría-García, M-A Rosales-Bérber, J Flores-Velázquez, A Pozos-Guillén
BACKGROUND: Congenital Heart Diseases (CHD) involves a wide range of pathological conditions, such as Pulmonary Atresia with Ventricle Septal Defect (PA/VSD). This disorder leads to the systemic circulation of oxygen-poor blood (cyanosis), with associated features and consequences in the oral cavity. MATERIAL AND METHODS: Using scoping review methodology for screening and article selection, the primary objectives of this paper were as follows: first, to pose a research question; second, to identify relevant studies in order to answer the research question; third, to select and retrieve the studies; fourth, to chart the critical data, and finally, to collate, summarize, and report the results from the most important articles on the dental management of children affected with PA/VSD...
June 18, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
https://www.readbyqxmd.com/read/28624249/the-role-of-3-d-heart-models-in-planning-and-executing-interventional-procedures
#4
REVIEW
Elena K Grant, Laura J Olivieri
Percutaneous interventions aimed at addressing congenital and structural heart disease are simultaneously becoming more common and more complex as time progresses. An increasing number of heart defects that had previously required open heart surgery can now be successfully addressed in the cardiac catheterization laboratory. Adequate preprocedural preparation for these novel, complex procedures is critical to ensure their success. Diagnostic data can be collected before the intervention and displayed in multiple formats during the procedure...
February 24, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28623384/the-role-of-cardiopulmonary-exercise-testing-for-decision-making-in-patients-with-repaired-tetralogy-of-fallot
#5
REVIEW
Frederic Dallaire, Rachel M Wald, Ariane Marelli
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. As a result of the surgical strategies employed at the time of initial repair, chronic pulmonary regurgitation (PR) is prevalent in this population. Despite sustained research efforts, patient selection and timing of pulmonary valve replacement (PVR) to address PR in young asymptomatic patients with repaired tetralogy of Fallot (rToF) remain a fundamental but as yet unanswered question in the field of congenital heart disease...
June 16, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28620608/a-path-to-implement-precision-child-health-cardiovascular-medicine
#6
REVIEW
Marlin Touma, Brian Reemtsen, Nancy Halnon, Juan Alejos, J Paul Finn, Stanley F Nelson, Yibin Wang
Congenital heart defects (CHDs) affect approximately 1% of live births and are a major source of childhood morbidity and mortality even in countries with advanced healthcare systems. Along with phenotypic heterogeneity, the underlying etiology of CHDs is multifactorial, involving genetic, epigenetic, and/or environmental contributors. Clear dissection of the underlying mechanism is a powerful step to establish individualized therapies. However, the majority of CHDs are yet to be clearly diagnosed for the underlying genetic and environmental factors, and even less with effective therapies...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28614866/critical-congenital-heart-disease-screening-in-nicu-need-for-revision-and-standardization
#7
Nithi Fernandes, Billie Short, Veena Manja, Satyan Lakshminrusimha
No abstract text is available yet for this article.
June 14, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28600089/invasive-hemodynamics-of-adult-congenital-heart-disease-from-shunts-to-coarctation
#8
REVIEW
Surendranath R Veeram Reddy, Alan W Nugent, Thomas M Zellers, V Vivian Dimas
Adults with congenital heart disease are a growing population with increasingly more complex disease, in large part due to improvements in delivery of care to the pediatric population. Cardiac catheterization is an integral component of diagnosis and management in these patients. Careful attention to detail and a thorough understanding of intracardiac hemodynamics are critical to performing complete diagnostic evaluations. This article outlines the most commonly encountered lesions with guidelines for invasive assessment to help guide further therapy...
July 2017: Interventional cardiology clinics
https://www.readbyqxmd.com/read/28588119/high-flow-nasal-cannula-utilization-in-pediatric-critical-care
#9
Kristen D Coletti, Dayanand N Bagdure, Linda K Walker, Kenneth E Remy, Jason W Custer
BACKGROUND: High-flow nasal cannula (HFNC) is increasingly utilized in pediatrics, delivering humidified air and oxygen for respiratory conditions causing hypoxia and distress. In the neonatal ICU, it has been associated with better tolerance, lower complications, and lower cost. Few data exist regarding indications for use and the epidemiology of disease/pathology that warrants HFNC in the pediatric ICU. METHODS: This study is a retrospective cohort study of patients admitted to a tertiary children's hospital pediatric ICU and placed on HFNC from October 1, 2011 to October 31, 2013...
June 6, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28583744/-pulmonary-revalvulation-and-rhythmologenic-risks-in-patients-with-repaired-tetralogy-of-fallot
#10
Rosario Pilato, Dominique Lacroix, Olivia Domanski, François Godart
Tetralogy of Fallot is a frequent congenital heart disease that has been repaired since the mid-1950s. The follow-up after repair is good despite a persistent risk of sudden death. The risk factors in long-term follow-up are advanced age at repair, hemodynamic status of the right ventricle, QRS duration≥180ms, left ventricular dysfunction, and existence of sustained or not ventricular tachycardia. In the presence of significant pulmonary regurgitation, it is necessary to perform revalvulation either by classic surgery or cardiac catheterization...
June 2, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28556366/a-role-for-primary-cilia-in-aortic-valve-development-and-disease
#11
Katelynn A Toomer, Diana Fulmer, Lilong Guo, Alex Drohan, Neal Peterson, Paige Swanson, Brittany Brooks, Rupak Mukherjee, Simon Body, Josh Lipschutz, Andy Wessels, Russell A Norris
BACKGROUND: Bicuspid aortic valve (BAV) disease is the most common congenital heart defect affecting 0.5-1.2% of the population and causes significant morbidity and mortality. Only a few genes have been identified in pedigrees and no single gene-model explains BAV inheritance, thus supporting a complex genetic network of interacting genes. However, patients with rare syndromic diseases that stem from alterations in the structure and function of primary cilia ("ciliopathies") exhibit BAV as a frequent cardiovascular finding, suggesting primary cilia may factor broadly in disease etiology...
May 27, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28542743/neuroimaging-cardiovascular-physiology-and-functional-outcomes-in-infants-with-congenital-heart-disease
#12
REVIEW
Nathalie H P Claessens, Christopher J Kelly, Serena J Counsell, Manon J N L Benders
This review integrates data on brain dysmaturation and acquired brain injury using fetal and neonatal magnetic resonance imaging (MRI), including the contribution of cardiovascular physiology to differences in brain development, and the relationship between brain abnormalities and subsequent neurological impairments in infants with congenital heart disease (CHD). The antenatal and neonatal period are critical for optimal brain development; the developing brain is particularly vulnerable to haemodynamic disturbances during this time...
May 19, 2017: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/28533037/epidemiology-of-live-born-infants-with-nonimmune-hydrops-fetalis-insights-from-a-population-based-dataset
#13
Martina A Steurer, Shabnam Peyvandi, Rebecca J Baer, Tippi MacKenzie, Ben C Li, Mary E Norton, Laura L Jelliffe-Pawlowski, Anita J Moon-Grady
OBJECTIVE: To evaluate the incidence, etiology, and 1-year mortality of nonimmune hydrops fetalis (NIHF) and to identify risk factors for mortality in a contemporary population-based dataset. STUDY DESIGN: The California Office of Statewide Health Planning and Development maintains a database linking maternal and infant hospital discharge, readmissions, and birth and death certificate date from 1 year before to 1 year after birth. We searched the database (2005-2012) for infants with NIHF (identified by the International Classification of Diseases, 9th Revision, Clinical Modification code)...
May 19, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28506601/a-flatline-lead-i-in-a-neonate-with-suspected-congenital-heart-disease
#14
Kristy Riniker, Luis Rigales, Irwin Hoffman
In a neonate with bradycardia and hypoxia, and clinical concern for critical congenital heart disease (CCHD), highly unusual ECG findings were present. These included a flatline Lead 1, and identical Leads 2, 3, and AVF. These abnormalities were due to bilateral arm to leg lead exchanges and not to heart disease. Complete cardiac workup including echocardiogram was normal.
April 21, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28504505/retinovascular-findings-in-newborns-with-critical-congenital-heart-disease-a-case-series
#15
Nurdan Fettah, Emrah U Kabatas, Vehbi Doğan, Aysegu L Zenciroğlu, Dilek Dilli, Elif Özyazıcı, Murat Koç, Serdar Beken, Arzu Dursun, Selmin Kandemir
Critical congenital heart disease (CCHD) may cause changes in retinal vasculature. However, there is lack of data in this issue in newborns. We evaluated retinovascular changes in a series of 43 newborn with CCHD. They were divided into 2 groups according to the type of CCHD; group 1 (n= 18): left obstructive CCHD and group 2 (n= 25): right obstructive CCHD. Retinovascular pathologies were detected in 21 patients (48.8%); it was more frequent in group 1 (p= 0.04). The most common pathology was retinal vascular tortuosity in 6 patients (33...
June 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28493526/real-time-mri-guidance-of-cardiac-interventions
#16
Adrienne E Campbell-Washburn, Mohammad A Tavallaei, Mihaela Pop, Elena K Grant, Henry Chubb, Kawal Rhode, Graham A Wright
Cardiac magnetic resonance imaging (MRI) is appealing to guide complex cardiac procedures because it is ionizing radiation-free and offers flexible soft-tissue contrast. Interventional cardiac MR promises to improve existing procedures and enable new ones for complex arrhythmias, as well as congenital and structural heart disease. Guiding invasive procedures demands faster image acquisition, reconstruction and analysis, as well as intuitive intraprocedural display of imaging data. Standard cardiac MR techniques such as 3D anatomical imaging, cardiac function and flow, parameter mapping, and late-gadolinium enhancement can be used to gather valuable clinical data at various procedural stages...
May 11, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28490416/development-and-assessment-of-an-e-learning-course-on-pediatric-cardiology-basics
#17
Ana Cristina Oliveira, Sandra Mattos, Miguel Coimbra
BACKGROUND: Early detection of congenital heart disease is a worldwide problem. This is more critical in developing countries, where shortage of professional specialists and structural health care problems are a constant. E-learning has the potential to improve capacity, by overcoming distance barriers and by its ability to adapt to the reduced time of health professionals. OBJECTIVE: The study aimed to develop an e-learning pediatric cardiology basics course and evaluate its pedagogical impact and user satisfaction...
May 10, 2017: JMIR Medical Education
https://www.readbyqxmd.com/read/28481830/dose-prescription-and-delivery-in-neonates-with-congenital-heart-diseases-treated-with-continuous-veno-venous-hemofiltration
#18
Zaccaria Ricci, Francesco Guzzi, Germana Tuccinardi, Luca Di Chiara, William Clark, Stuart L Goldstein, Claudio Ronco
OBJECTIVES: Renal replacement therapy may be required for acute kidney injury treatment in neonates with complex cardiac conditions. Continuous veno-venous hemofiltration is applied safely in this population but no published recommendations for dose prescription in neonates currently exist. The aim of our study was to evaluate the effects of a relatively small dialysis dose on critically ill neonates. DESIGN: Retrospective analysis of clinical charts. SETTING: Pediatric Cardiac ICU...
May 5, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28477319/transition-of-care-in-congenital-heart-disease-ensuring-the-proper-handoff
#19
REVIEW
Angela Lee, Barbara Bailey, Geraldine Cullen-Dean, Sandra Aiello, Joanne Morin, Erwin Oechslin
BACKGROUND: With great advances in medical and surgical care, most congenital heart disease patients are living in to adulthood and require lifelong surveillance and expert care for adult onset complications. Care lapse and lack of successful transfer from pediatric to adult care put young adults at risk for increased morbidity and premature death. Hence, transition and transfer from pediatric to adult care is a crucial and critical process to provide access to specialized care and lifelong surveillance...
June 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28477067/erratum-to-critical-congenital-heart-disease-newborn-screening-implementation-lessons-learned
#20
Monica R McClain, John S Hokanson, Regina Grazel, Kim Van Naarden Braun, Lorraine F Garg, Michelle R Morris, Kathleen Moline, Keri Urquhart, Amy Nance, Harper Randall, Marci K Sontag
No abstract text is available yet for this article.
May 5, 2017: Maternal and Child Health Journal
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