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https://www.readbyqxmd.com/read/28537937/dexmedetomidine-the-new-all-in-one-drug-in-paediatric-anaesthesia
#1
Cedric E Sottas, Brian J Anderson
PURPOSE OF REVIEW: Dexmedetomidine is a drug with sedative, anxiolytic, sympatholytic and analgesic properties, which is finding widespread practice in paediatric anaesthesia and related practices. The present review summarizes its pharmacology and current experience with the drug. RECENT FINDINGS: Dexmedetomidine is proving useful in many diverse areas in paediatric anaesthesia where its sedative properties are useful for premedication, fibreoptic intubation and radiologic procedures...
May 22, 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28537936/zidovudine-use-in-pregnancy-and-congenital-malformations-meta-analysis-and-bayesian-incorporation-of-medicaid-data
#2
Kathryn Rough, Jenny W Sun, George R Seage, Paige L Williams, Krista F Huybrechts, Brian T Bateman, Sonia Hernandez-Diaz
OBJECTIVE: There is inconsistent evidence that zidovudine use during pregnancy increases overall, cardiac, and male genital malformations. DESIGN: We conducted a systematic review and meta-analysis of zidovudine use and malformations and, using Bayesian methods, combined it with data from a cohort study of mother-infant pairs in the nationwide Medicaid Analytic eXtract (MAX). METHODS: Using MAX data (2000-2010), we identified pregnant women with HIV treated with antiretroviral therapy (ART)...
May 23, 2017: AIDS
https://www.readbyqxmd.com/read/28534239/analysis-of-screening-electrocardiogram-for-the-subcutaneous-defibrillator-in-adults-with-congenital-heart-disease
#3
Vincent C Thomas, Mark Peterson, Martin McDaniel, Humberto Restrepo, Abraham Rothman, Amit Jain
Candidates for the subcutaneous implantable cardioverter-defibrillator (S-ICD) are screened using an electrocardiogram (S-ECG) tool to measure appropriate detection. We sought to define the S-ICD candidacy of congenital heart disease patients using the S-ECG tool. We also analyzed the reliability of the (S-ECG) tool between measurers in this population. Patients above the age of 12 and with a diagnosis associated with either a higher incidence of cardiac arrest or vascular access challenges were asked to undergo screening...
May 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28533990/anomalous-origin-of-right-coronary-artery-originating-from-the-pulmonary-trunk-arcapa-an-incidental-finding-in-a-patient-presenting-with-chest-pain
#4
Pragathi Balakrishna, Michael Illovsky, Youssef M Al-Saghir, Abdul M Minhas
Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare congenital coronary anomaly with an estimated prevalence of 0.002%. Most patients are asymptomatic and the anomaly is detected incidentally during evaluation for other problems. Occasionally, ARCAPA may lead to myocardial ischemia and/or sudden cardiac arrest. We present a case of a 55-year-old female with a history of hypertension who presented to the emergency department with intermittent chest discomfort for three days...
April 17, 2017: Curēus
https://www.readbyqxmd.com/read/28527921/drug-induced-fatal-arrhythmias-acquired-long-qt-and-brugada-syndromes
#5
REVIEW
Isik Turker, Tomohiko Ai, Hideki Itoh, Minoru Horie
Since the early 1990s, the concept of primary "inherited" arrhythmia syndromes or ion channelopathies has evolved rapidly as a result of revolutionary progresses made in molecular genetics. Alterations in genes coding for membrane proteins such as ion channels or their associated proteins responsible for the generation of cardiac action potentials (AP) have been shown to cause specific malfunctions which eventually lead to cardiac arrhythmias. These arrhythmic disorders include congenital long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, short QT syndrome, progressive cardiac conduction disease, etc...
May 17, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28525615/embolic-foreign-material-in-the-central-nervous-system-of-pediatric-autopsy-patients-with-instrumented-heart-disease
#6
Matthew Torre, Mirna Lechpammer, Vera Paulson, Sanjay Prabhu, Audrey C Marshall, Amy L Juraszek, Robert F Padera, Elizabeth A Bundock, Sara O Vargas, Rebecca D Folkerth
Upon detection of foreign-body embolization to the central nervous system (CNS) following a specific invasive cardiovascular procedure in 1 autopsied child, we undertook a quality assurance analysis to determine whether other patients had had similar events. Autopsies of all infants and children with history of cardiac catheterization, heart surgery on cardiopulmonary bypass, and/or extracorporeal membrane oxygenation over a 5-year period at a single tertiary care institution were reviewed for light-microscopic evidence of foreign material...
May 19, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28521893/pulmonary-hypertension-in-infants-children-and-young-adults
#7
REVIEW
Georg Hansmann
Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure...
May 23, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28521630/isolated-ventricular-noncompaction-cardiomyopathy-presenting-as-fetal-hydrops-at-24-weeks-gestation
#8
Jane E Armes, Lisa Squires, Rohan Lourie, Mark Williams, Renee Gallagher, Gareth Price, Andrew Stubbs, Sigrid Ma Swagemakers, Peter J van der Spek, James Harraway, Joseph Thomas, Deon J Venter
Ventricular noncompaction cardiomyopathy is a rare form of congenital cardiomyopathy with increasing evidence of genetic etiology, especially when presenting in childhood. Fetal presentation is rare. We describe a case of fetal hydrops, presenting at 24 weeks gestation and leading to intrapartum death at 26 weeks gestation. Autopsy examination revealed characteristic features of left ventricular noncompaction. A genetic analysis identified a constellation of variants of unknown significance in MYH6, TNNC1, and MYBPC3, genes known to be important in sarcomeric function...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28521042/induced-pluripotent-stem-cell-modelling-of-hlhs-underlines-the-contribution-of-dysfunctional-notch-signalling-to-impaired-cardiogenesis
#9
Chunbo Yang, Yaobo Xu, Min Yu, David Lee, Sameer Alharti, Nicola Hellen, Noor Ahmad Shaik, Babajan Banaganapalli, Hussein Ali Mohamoud Sheikh, Elango Ramu, Stefan Przyborski, Gennadiy Tenin, Simon Williams, John O'Sullivan, Osman O Al-Radi, Jameel Atta, Sian E Harding, Bernard Keavney, Majlinda Lako, Lyle Armstrong
Hypoplastic left heart syndrome (HLHS) is among the most severe forms of congenital heart disease. Although the consensus view is that reduced flow through the left heart during development is a key factor in the development of the condition, the molecular mechanisms leading to hypoplasia of left heart structures are unknown. We have generated induced pluripotent stem cells (iPSC) from five HLHS patients and two unaffected controls, differentiated these to cardiomyocytes and identified reproducible in vitro cellular and functional correlates of the HLHS phenotype...
May 17, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28518124/direct-re-implantation-of-left-coronary-artery-into-the-aorta-in-adults-with-anomalous-origin-of-left-coronary-artery-from-the-pulmonary-artery-alcapa
#10
Reza Tavakoli, Peiman Jamshidi, Nassrin Yamani, Max Gassmann
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly which is one of leading causes of myocardial ischemia and infarction in children. If left untreated, it results in a 90% mortality rate in the first year of life. In patients who survive to the adulthood, the coronary steal phenomenon and retrograde left-sided coronary flow provide a substrate for chronic subendocardial ischemia, which may lead to left ventricular dysfunction, ischemic mitral regurgitation, malignant ventricular arrhythmias, and sudden cardiac death...
April 24, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28515851/blood-conservation-pediatric-cardiac-surgery-in-all-ages-and-complexity-levels
#11
Mohsen Karimi, Jill M Sullivan, Carrie Linthicum, Anil Mathew
AIM: To demonstrate the feasibility of blood conservation methods and practice across all ages and risk categories in congenital cardiac surgery. METHODS: We retrospectively analyzed a collected database of 356 patients who underwent cardiac surgery using cardiopulmonary bypass (CPB) from 2010-2015. The patients were grouped into blood conservation (n = 138) and non-conservation (n = 218) groups and sub-grouped based on their ages and procedural complexity scores...
April 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28515759/department-of-pediatric-cardiac-surgery-in-gdansk-in-its-new-location-previous-activity-and-perspectives-for-development
#12
Ireneusz Haponiuk, Maciej Chojnicki, Mariusz Steffens, Radosław Jaworski, Aneta Szofer-Sendrowska, Konrad Paczkowski, Ewelina Kwaśniak, Anna Romanowicz, Wiktor Szymanowicz, Katarzyna Gierat-Haponiuk
The Department of Pediatric Cardiac Surgery in Gdansk is the only pediatric cardiac surgery center in northern Poland providing comprehensive treatment to children with congenital heart defects. The Department of Pediatric Cardiac Surgery in Gdansk currently offers a full spectrum of advanced procedures of modern cardiac surgery and interventional cardiology dedicated to patients from infancy to adolescence. January 19, 2016 marked the official opening of its new location.
March 2017: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/28515589/spectrum-of-pulmonary-valve-morphology-and-its-relationship-to-pulmonary-trunk-in-tetralogy-of-fallot
#13
Binita R Chacko, George K Chiramel, Leena R Vimala, Devi A Manuel, Elizabeth Joseph, K Reka
BACKGROUND: Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail. AIMS: The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk...
January 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28515370/acute-kidney-injury-following-cardiopulmonary-bypass-in-children%C3%A3-risk-factors-and-outcomes
#14
Seon Hwa Lee, Soo-Jin Kim, Hyung Joong Kim, Jae Sung Son, Ran Lee, Tae Gyoon Yoon
BACKGROUND: Acute kidney injury (AKI) is the most common and most serious complication following heart surgery. We aimed to determine the prevalence of, and risk factors for, AKI following pediatric cardiac surgery.Methods and Results:We retrospectively analyzed 135 patients aged ≤18 years who underwent cardiac surgery for congenital heart defects; by RACHS-1 category, 58 patients (43%) had an operative risk score ≥3. AKI was defined and classified using the pediatric pRIFLE criteria (Pediatric Risk, Injury, Failure, Loss, and End-stage Kidney Disease); 19 patients (14...
May 17, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28515113/force-sensing-catheters-during-pediatric-radiofrequency-ablation-the-federation-study
#15
Aarti S Dalal, Hoang H Nguyen, Tammy Bowman, George F Van Hare, Jennifer N Avari Silva
BACKGROUND: Based on data from studies of atrial fibrillation ablations, optimal parameters for the TactiCath (TC; St. Jude Medical, Inc) force-sensing ablation catheter are a contact force of 20 g and a force-time integral of 400 g·s for the creation of transmural lesions. We aimed to evaluate TC in pediatric and congenital heart disease patients undergoing ablation. METHODS AND RESULTS: Comprehensive chart and case reviews were performed from June 2015 to March 2016...
May 17, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28514670/radiation-safety-in-children-with-congenital-and-acquired-heart-disease-a-scientific-position-statement-on-multimodality-dose-optimization-from-the-image-gently-alliance
#16
REVIEW
Kevin D Hill, Donald P Frush, B Kelly Han, Brian G Abbott, Aimee K Armstrong, Robert A DeKemp, Andrew C Glatz, S Bruce Greenberg, Alexander Sheldon Herbert, Henri Justino, Douglas Mah, Mahadevappa Mahesh, Cynthia K Rigsby, Timothy C Slesnick, Keith J Strauss, Sigal Trattner, Mohan N Viswanathan, Andrew J Einstein
There is a need for consensus recommendations for ionizing radiation dose optimization during multi-modality medical imaging in children with congenital and acquired heart disease (CAHD). These children often have complex diseases and may be exposed to a relatively high cumulative burden of ionizing radiation from medical imaging procedures including cardiac computed tomography, nuclear cardiology studies and fluoroscopically guided diagnostic and interventional catheterization and electrophysiology procedures...
May 17, 2017: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28514318/cerebral-oxygen-saturation-in-children-with-congenital-heart-disease-and-chronic-hypoxemia
#17
Barry D Kussman, Peter C Laussen, Paul B Benni, Francis X McGowan, Doff B McElhinney
BACKGROUND: Increased hemoglobin (Hb) concentration accompanying hypoxemia is a compensatory response to maintain tissue oxygen delivery. Near infrared spectroscopy (NIRS) is used clinically to detect abnormalities in the balance of cerebral tissue oxygen delivery and consumption, including in children with congenital heart disease (CHD). Although NIRS-measured cerebral tissue O2 saturation (ScO2) correlates with arterial oxygen saturation (SaO2), jugular bulb O2 saturation (SjbO2), and Hb, little data exist on the interplay between these factors and cerebral O2 extraction (COE)...
May 12, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28513458/cofilin-a-protein-controlling-dynamics-of-actin-filaments
#18
Zofia Ostrowska, Joanna Moraczewska
Cofilins are evolutionary conserved proteins present in all Eukaryotic cells. Their primary function is dynamic reorganization of actin cytoskeleton. Two cofilin isoforms are known: cofilin 1, present in all studied non-muscle cells and in embryonic muscle cells, and cofilin 2, which dominates in mature skeletal and cardiac muscles. Polypeptide chains of both isoforms fold into a structure homological to a conservative ADF (actin depolymerizing factor) domain, which is characteristic of actin depolymerizing factor...
May 5, 2017: Postȩpy Higieny i Medycyny Doświadczalnej
https://www.readbyqxmd.com/read/28511778/elevated-cranial-ultrasound-resistive-indices-are-associated-with-improved-neurodevelopmental-outcomes-one-year-after-pediatric-cardiac-surgery-a-single-center-pilot-study
#19
Christopher L Jenks, Ana Hernandez, Peter L Stavinoha, Michael C Morris, Fenghua Tian, Hanli Liu, Parvesh Garg, Joseph M Forbess, Joshua Koch
OBJECTIVE: To determine if a non-invasive, repeatable test can be used to predict neurodevelopmental outcomes in patients with congenital heart disease. METHODS: This was a prospective study of pediatric patients less than two months of age undergoing congenital heart surgery at the Children's Health Children's Medical Center at Dallas. Multichannel near-infrared spectroscopy (NIRS) was utilized during the surgery, and ultrasound (US) resistive indices (RI) of the major cranial vessels were obtained prior to surgery, immediately post-operatively, and prior to discharge...
May 13, 2017: Heart & Lung: the Journal of Critical Care
https://www.readbyqxmd.com/read/28511407/gerbode-ventricular-septal-defect-a-rare-cardiac-anomaly-associated-with-genetic-variants-in-indian-population-a-case-series
#20
Yashvanthi Borkar, Krishnananda Nayak, Ranjan K Shetty, Gopalakrishna Bhat, Rajasekhar Moka
Gerbode defects are rare Ventricular Septal Defects (VSD) constituting approximately one percent cases of congenital heart diseases. The genetic predispositions towards the Gerbode Defect (GD) have remained an unexplored area of study till date. We investigated the genotype-phenotype correlation in patients with Gerbode VSD. Molecular genetic study on Sanger sequencing and subsequent data analysis showed that the contributing sequence variations in the NKX2-5, GATA4 and TBX5 gene lies in one of the highly conserved regions and this region is responsible for encoding a functional protein...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
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