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Critical congenital heart disease

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https://www.readbyqxmd.com/read/28212589/a-multicenter-initiative-for-critical-congenital-heart-disease-newborn-screening-in-texas-neonatal-intensive-care-units
#1
Alice Gong, Charleta Guillory, Liza Creel, Judith Ellen Livingtson, Tiffany M McKee-Garrett, Regine Fortunov
Objective The objective of this study was to implement a strategy for critical congenital heart disease (CCHD) newborn screening in the neonatal intensive care unit (NICU). Design A NICU-specific curriculum, screening algorithm, slide presentations, and templates of orders, policies, and procedures were developed into a toolkit for training NICU personnel. Screening was conducted on first and second screen pre- and postductal oxygen saturations (SpO2) on newborns admitted or transferred to the NICU. Results We trained 347 NICU personnel in 13 Texas hospitals, representing rural, suburban, and metropolitan settings...
February 17, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28212053/transthoracic-echocardiography-pitfalls-and-limitations-as-delineated-at-cardiac-ct-and-mr-imaging
#2
Sachin B Malik, Natalie Chen, Rex A Parker, Joe Y Hsu
Transthoracic echocardiography ( TTE transthoracic echocardiography ) is a critical tool in the field of clinical cardiology. It often serves as one of the first-line imaging modalities in the evaluation of cardiac disease owing to its low cost, portability, widespread availability, lack of ionizing radiation, and ability to evaluate both anatomy and function of the heart. Consequently, a large majority of patients undergoing a cardiac computed tomography (CT) or magnetic resonance (MR) imaging examination will have a TTE transthoracic echocardiography available for review...
February 17, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28210520/synchronous-aberrant-cerebellar-and-opercular-development-in-fetuses-and-neonates-with-congenital-heart-disease-correlation-with-early-communicative-neurodevelopmental-outcomes-initial-experience
#3
A Wong, T Chavez, S O'Neil, J Votava-Smith, D Miller, S delCastillo, A Panigrahy, L Paquette
Patients with congenital heart disease (CHD) demonstrate multidomain cognitive delays. Cingulo-opercular and cerebellar brain networks are critical to language functions. This is a description of our initial experience aiming to identify an anatomic correlate for CHD patients with expressive language delays. Fetal CHD patients, prospectively enrolled, underwent serial fetal (1.5T), postnatal pre- and postoperative (3T) MRI. Non-CHD patients were enrolled retrospectively from the same epoch. Comparable fetal and neonatal T2 contrast was used for manual linear cross-sectional measurement...
January 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/28195132/analysis-of-craniocardiac-malformations-in-xenopus-using-optical-coherence-tomography
#4
Engin Deniz, Stephan Jonas, Michael Hooper, John N Griffin, Michael A Choma, Mustafa K Khokha
Birth defects affect 3% of children in the United States. Among the birth defects, congenital heart disease and craniofacial malformations are major causes of mortality and morbidity. Unfortunately, the genetic mechanisms underlying craniocardiac malformations remain largely uncharacterized. To address this, human genomic studies are identifying sequence variations in patients, resulting in numerous candidate genes. However, the molecular mechanisms of pathogenesis for most candidate genes are unknown. Therefore, there is a need for functional analyses in rapid and efficient animal models of human disease...
February 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28150025/initial-field-test-of-a-cloud-based-cardiac-auscultation-system-to-determine-murmur-etiology-in-rural-china
#5
Lee Pyles, Pouya Hemmati, J Pan, Xiaoju Yu, Ke Liu, Jing Wang, Andreas Tsakistos, Bistra Zheleva, Weiguang Shao, Quan Ni
A system for collection, distribution, and long distant, asynchronous interpretation of cardiac auscultation has been developed and field-tested in rural China. We initiated a proof-of-concept test as a critical component of design of a system to allow rural physicians with little experience in evaluation of congenital heart disease (CHD) to obtain assistance in diagnosis and management of children with significant heart disease. The project tested the hypothesis that acceptable screening of heart murmurs could be accomplished using a digital stethoscope and internet cloud transmittal to deliver phonocardiograms to an experienced observer...
February 2, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28148729/psychiatric-disorders-in-adolescents-with-single-ventricle-congenital-heart-disease
#6
David R DeMaso, Johanna Calderon, George A Taylor, Jennifer E Holland, Christian Stopp, Matthew T White, David C Bellinger, Michael J Rivkin, David Wypij, Jane W Newburger
BACKGROUND AND OBJECTIVES: Mental health outcomes for survivors of critical congenital heart disease (CHD) remain under-investigated. We sought to examine psychiatric disorders and psychosocial functioning in adolescents with single ventricle CHD and to explore whether patient-related risk factors predict dysfunction. METHODS: This cohort study recruited 156 adolescents with single ventricle CHD who underwent the Fontan procedure and 111 healthy referents. Participants underwent comprehensive psychiatric evaluation including a clinician-rated psychiatric interview and parent- and self-report ratings of anxiety, disruptive behavior, including attention-deficit/hyperactivity disorder (ADHD), and depressive symptoms...
February 1, 2017: Pediatrics
https://www.readbyqxmd.com/read/28139441/normative-values-of-aortic-arch-structures-in-premature-infants
#7
Elles J Dijkema, Mirella C Molenschot, Johannes M P J Breur, Willem B de Vries, Martijn G Slieker
BACKGROUND: Aortic arch abnormalities represent 5% to 8% of all congenital heart disease. Measurements of the aortic arch dimensions on two-dimensional echocardiographic images remain of critical importance in the diagnosis of aortic arch pathology. To define aortic hypoplasia or coarctation, measured dimensions must be compared with normal values. Normal values have been described for children of all ages in earlier studies. However, normative data for premature infants are not yet available...
January 27, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28129863/a-national-call-to-action-improving-the-detection-of-critical-congenital-heart-disease
#8
EDITORIAL
Kevin C Harris
No abstract text is available yet for this article.
February 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28123459/role-of-micrornas-in-cardiac-development-and-disease
#9
Jing Tian, Xinjiang An, Ling Niu
Heart disease-related deaths are the highest in most societies and congenital heart diseases account for approximately 40% of prenatal deaths and over 20% of mortality in the first few months after birth. Congenital heart disease affects approximately 1% of all newborns and is the causative factor for more deaths within the first year of life as compared to all other genetic defects. Advances in treatment approaches increased life expectancy and led to an expansion of adult population with clinical manifestation of congenital heart defects in up to 90% of the children born with congenital heart diseases...
January 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28121514/genetic-drivers-of-kidney-defects-in-the-digeorge-syndrome
#10
Esther Lopez-Rivera, Yangfan P Liu, Miguel Verbitsky, Blair R Anderson, Valentina P Capone, Edgar A Otto, Zhonghai Yan, Adele Mitrotti, Jeremiah Martino, Nicholas J Steers, David A Fasel, Katarina Vukojevic, Rong Deng, Silvia E Racedo, Qingxue Liu, Max Werth, Rik Westland, Asaf Vivante, Gabriel S Makar, Monica Bodria, Matthew G Sampson, Christopher E Gillies, Virginia Vega-Warner, Mariarosa Maiorana, Donald S Petrey, Barry Honig, Vladimir J Lozanovski, Rémi Salomon, Laurence Heidet, Wassila Carpentier, Dominique Gaillard, Alba Carrea, Loreto Gesualdo, Daniele Cusi, Claudia Izzi, Francesco Scolari, Joanna A E van Wijk, Adela Arapovic, Mirna Saraga-Babic, Marijan Saraga, Nenad Kunac, Ali Samii, Donna M McDonald-McGinn, Terrence B Crowley, Elaine H Zackai, Dorota Drozdz, Monika Miklaszewska, Marcin Tkaczyk, Przemyslaw Sikora, Maria Szczepanska, Malgorzata Mizerska-Wasiak, Grazyna Krzemien, Agnieszka Szmigielska, Marcin Zaniew, John M Darlow, Prem Puri, David Barton, Emilio Casolari, Susan L Furth, Bradley A Warady, Zoran Gucev, Hakon Hakonarson, Hana Flogelova, Velibor Tasic, Anna Latos-Bielenska, Anna Materna-Kiryluk, Landino Allegri, Craig S Wong, Iain A Drummond, Vivette D'Agati, Akira Imamoto, Jonathan M Barasch, Friedhelm Hildebrandt, Krzysztof Kiryluk, Richard P Lifton, Bernice E Morrow, Cecile Jeanpierre, Virginia E Papaioannou, Gian Marco Ghiggeri, Ali G Gharavi, Nicholas Katsanis, Simone Sanna-Cherchi
Background The DiGeorge syndrome, the most common of the microdeletion syndromes, affects multiple organs, including the heart, the nervous system, and the kidney. It is caused by deletions on chromosome 22q11.2; the genetic driver of the kidney defects is unknown. Methods We conducted a genomewide search for structural variants in two cohorts: 2080 patients with congenital kidney and urinary tract anomalies and 22,094 controls. We performed exome and targeted resequencing in samples obtained from 586 additional patients with congenital kidney anomalies...
25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28097194/the-mystery-of-the-z-score
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
February 2017: 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
#19
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
#20
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
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