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Critical congenital heart disease

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https://www.readbyqxmd.com/read/28807423/coping-with-the-stress-in-the-cardiac-intensive-care-unit-can-mindfulness-be-the-answer
#1
Nadya Golfenshtein, Janet A Deatrick, Amy J Lisanty, Barbara Medoff-Cooper
BACKGROUND: Mothers of infants with complex congenital heart disease are exposed to increased stress which has been associated with numerous adverse health outcomes. The coping mechanisms these mothers use critically effect the familial illness adaptation and most likely infant outcomes. Currently no data-based strategies have been developed for mothers to facilitate their coping, and proactively promote their adaptation in the critical care settings. A potential strategy is mindfulness which is currently used in other clinical settings with stress-reduction effects...
August 11, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28766834/transseptal-puncture-using-surgical-electrocautery-in-children-and-adults-with-and-without-complex-congenital-heart-disease
#2
Srinath T Gowda, Athar M Qureshi, Daniel Turner, Nitin Madan, Justin Weigand, Richard Lorber, Harinder R Singh
BACKGROUND: Atrial transseptal puncture (TSP) for cardiac catheterization procedures remain challenging in children and adults with complex congenital heart disease (CHD). OBJECTIVES: We sought to evaluate our experience using radiofrequency (RF) current via surgical electrocautery needle for TSP to facilitate diagnostic and interventional procedures. METHODS: Retrospective chart review of all patients (pts) who underwent TSP using RF energy (10-25 W) via surgical electrocautery from three centers from January 2011 to January 2017 were evaluated...
August 2, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28765741/experience-with-mechanical-circulatory-support-for-medically-intractable-low-cardiac-output-in-a-pediatric-intensive-care-unit
#3
Jung Bin Park, Jae Gun Kwak, Hong-Gook Lim, Woong-Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28746973/prenatal-maternal-hyperoxygenation-testing-and-implications-for-critical-care-delivery-planning-among-fetuses-with-congenital-heart-disease-early-experience
#4
David N Schidlow, Mary T Donofrio
No abstract text is available yet for this article.
July 26, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28745539/congenital-heart-disease-and-genetic-syndromes-new-insights-into-molecular-mechanisms
#5
Giulio Calcagni, Marta Unolt, Maria Cristina Digilio, Anwar Baban, Paolo Versacci, Marco Tartaglia, Antonio Baldini, Bruno Marino
Advances in genetics allowed a better definition of the role of specific genetic background in the etiology of syndromic congenital heart defects (CHDs). The identification of a number of disease genes responsible for different syndromes have led to the identification of several transcriptional regulators and signaling transducers and modulators that are critical for heart morphogenesis. Understanding the genetic background of syndromic CHDs allowed a better characterization of the genetic basis of non-syndromic CHDs...
August 3, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28725922/genetic-testing-protocol-reduces-costs-and-increases-rate-of-genetic-diagnosis-in-infants-with-congenital-heart-disease
#6
Gabrielle C Geddes, Donald Basel, Peter Frommelt, Aaron Kinney, Michael Earing
Genetic testing is routinely performed on infants with critical congenital heart disease (CHD). This project reviewed the effect of implementing a genetic testing protocol in this population. Charts of infants with critical CHD were reviewed for genetic testing and results across two time periods: the time before implementation of a genetic testing protocol (pre-protocol) and the time after implementation (post-protocol). The use of karyotype, 22q11.2 Deletion testing, and chromosomal microarray were compared across these two time periods...
July 19, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28719069/inaccuracy-of-a-continuous-arterial-pressure-waveform-monitor-when-used-for-congenital-cardiac-catheterization
#7
Michael D Seckeler, Katri Typpo, Jendar Deschenes, Ruth Higgins, Ricardo Samson, Peter Lichtenthal
OBJECTIVE: To determine the accuracy of a continuous cardiac output monitor (FloTrac sensor) for measuring cardiac index in children with congenital heart disease undergoing cardiac catheterization. Cardiac index is a critical hemodynamic parameter measured during catheterizations in children with congenital heart disease. This has been challenging to measure accurately and many clinicians rely on predictive equations for calculating cardiac index. DESIGN: Prospective, nonrandomized trial...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28711963/transfer-of-neonates-with-critical-congenital-heart-disease-within-a-regionalized-network
#8
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/28696220/prevention-of-sudden-cardiac-death-in-adults-with-congenital-heart-disease-do-the-guidelines-fall-short
#9
Jim T Vehmeijer, Zeliha Koyak, Werner Budts, Louise Harris, Candice K Silversides, Erwin N Oechslin, Berto J Bouma, Aeilko H Zwinderman, Barbara J M Mulder, Joris R de Groot
BACKGROUND: Sudden cardiac death (SCD) is a major cause of mortality in adult congenital heart disease (ACHD) patients. SCD may be prevented by implantable cardioverter-defibrillator (ICD) implantation, but patient stratification remains troublesome. The 2014 Consensus Statement on Arrhythmias in ACHD patients and the 2015 European Society of Cardiology Guidelines specified recommendations for ICD implantation in ACHD patients for the first time. We assess the discriminative ability of these ICD recommendations for SCD in ACHD patients...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28680604/atrial-flutter-in-a-patient-with-atrial-septal-defect-and-anomalous-venous-drainage-unusual-approach-for-ablation
#10
Ivo Roca-Luque, Nuria Rivas, Laura Dos, Jaume Francisco, Jordi Pérez-Rodon, Antònia Pijuan, David Garcia-Dorado, Àngel Moya
Atrial flutter ablation in CHD (Congenital Heart Disease) patients is a challenging procedure because of the possibility of multiple circuits. Electroanatomical mapping and pacing maneuvers are crucial to determine critical isthmus. Moreover, vascular abnormalities and residual cardiac defects need to be known before the ablation to decide the better strategy for ablation.
July 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28672762/critical-congenital-heart-disease-screening-with-a-pulse-oximetry-in-neonates
#11
Şahin Hamilçıkan, Emrah Can
OBJECTIVE: To compare the results of pulse oximetry screening for critical congenital heart disease (CCHD) in newborn infants performed at <24 h and >24 h following. METHOD: Measurements were taken for each group at <24 h and >24 h following birth. Echocardiography was performed if the SpO2 readings remained abnormal results. RESULTS: A total of 4518 newborns were included in this prospective descriptive study. Of these, 2484 (60...
June 27, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28669924/histone-h3-lysine-4-methyltransferase-kmt2d
#12
REVIEW
Eugene Froimchuk, Younghoon Jang, Kai Ge
Histone-lysine N-methyltransferase 2D (KMT2D), also known as MLL4 and MLL2 in humans and Mll4 in mice, belongs to a family of mammalian histone H3 lysine 4 (H3K4) methyltransferases. It is a large protein over 5500 amino acids in size and is partially functionally redundant with KMT2C. KMT2D is widely expressed in adult tissues and is essential for early embryonic development. The C-terminal SET domain is responsible for its H3K4 methyltransferase activity and is necessary for maintaining KMT2D protein stability in cells...
September 5, 2017: Gene
https://www.readbyqxmd.com/read/28668447/effect-of-cryopreservation-on-viability-and-growth-efficiency-of-stromal-epithelial-cells-derived-from-neonatal-human-thymus
#13
Valentin P Shichkin, Oleksandr I Gorbach, Olga A Zuieva, Nataliia I Grabchenko, Irina A Aksyonova, Boris M Todurov
The thymus is the major site of T lymphocyte generation and so is critical for a functional adaptive immune system. Since, thymectomy is a component of neonatal surgery for congenital heart diseases, it provides great potential for collection and storage of thymic tissue for autologous transplantation. However, specific investigation into the optimum parameters for thymic tissue cryopreservation have not been conducted. In this research, we evaluated the effect of different cryoprotective media compositions, which included penetrating (Me2SO, glycerol) and non-penetrating (dextran-40, sucrose, hydroxyethyl starch) components, on the viability and functionality of frozen-thawed human thymic samples to select an optimal cryoprotective medium suitable for long-term storage of thymic tissue and a stromal-epithelial enriched population...
June 28, 2017: Cryobiology
https://www.readbyqxmd.com/read/28668199/chronic-respiratory-failure-utilization-of-a-pediatric-specialty-integrated-care-program
#14
Robert J Graham, Michael L McManus, Angie Mae Rodday, Ruth Ann Weidner, Susan K Parsons
OBJECTIVE: Describe utilization and satisfaction in a specialty integrated care program for children with severe, chronic respiratory insufficiency (CRI). SUBJECTS: Enrollees of the Critical Care, Anesthesia, Perioperative Extension (CAPE) and Home Ventilation Program. METHODS: Children with CRI received home visits, care coordination, and "on-demand" 24/7 access to physicians. Program activity and outcomes were recorded for 3 years using an adapted Care Coordination Measurement Tool© version...
March 2017: Healthcare
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
#15
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)...
September 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
#16
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
#17
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...
July 1, 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
#18
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
#19
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
#20
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
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