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Critical Congenital heart

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https://www.readbyqxmd.com/read/28506601/a-flatline-lead-i-in-a-neonate-with-suspected-congenital-heart-disease
#1
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
#2
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
#3
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
#4
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/28489314/unclassifiable-pattern-of-hypopigmentation-in-a-patient-with-mosaic-partial-12p-tetrasomy-without-pallister-killian-syndrome
#5
Viola Alesi, Maria L Dentici, Fabrizia Restaldi, Valeria Orlando, Maria T Liambo, Chiara Calacci, Rossella Capolino, Maria C Digilio, May El Hachem, Antonio Novelli, Andrea Diociaiuti, Bruno Dallapiccola
Pallister-Killian syndrome (PKS-#OMIM601803) is a multisystem developmental disorder typically due to the presence of an aneuploidy cell line, consisting of a supernumerary tetrasomic chromosomal marker (SCM) arisen from the short arm of chromosome 12 (12p isochromosome). The clinical phenotype, which is strictly related to the percentage and tissue distribution of aneuploid cells, is characterized by craniofacial dysmorphisms, pigmentary skin anomalies, limb shortening, congenital heart defects, diaphragmatic hernia, hypotonia, intellectual disability, and epilepsy...
May 10, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28483478/left-aberrant-subclavian-artery-systematic-study-in-adult-patients
#6
Paweł Tyczyński, Ilona Michałowska, Rafał Wolny, Piotr Dobrowolski, Hubert Łazarczyk, Justyna Rybicka, Piotr Hoffman, Adam Witkowski
BACKGROUND: Left aberrant subclavian artery (LASA), is a type of right aortic arch (RAA) branching, which takes-off distally to the right subclavian artery and usually crosses behind the esophagus to the left upper limb. Taking into account the rarity of RAA, LASA is much more rarely seen than the right aberrant subclavian artery (RASA) originating from the left aortic arch. However, RAA may be associated with much more frequent presence of LASA, than left aortic arch with RASA. Anatomical LASA characteristics were not described up to date...
April 25, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28481830/dose-prescription-and-delivery-in-neonates-with-congenital-heart-diseases-treated-with-continuous-veno-venous-hemofiltration
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/28460704/continuous-capnography-in-pediatric-intensive-care
#10
REVIEW
Christine M Riley
Capnography or end-tidal carbon dioxide (Etco2) monitoring has a variety of uses in the pediatric intensive care setting. The ability to continuously measure exhaled carbon dioxide can provide vital information about airway, breathing, and circulation in critically ill pediatric patients. Capnography has diagnosis-specific applications for pediatric patients with congenital heart disease, reactive airway disease, neurologic emergencies, and metabolic derangement. This modality allows for noninvasive monitoring and has become the standard of care...
June 2017: Critical Care Nursing Clinics of North America
https://www.readbyqxmd.com/read/28441821/-assessment-of-undiagnosed-critical-congenital-heart-disease-before-discharge-from-the-maternity-hospital
#11
Q M Zhao, F Liu, L Wu, M Ye, B Jia, X J Ma, G Y Huang
Objective: Undiagnosed critical congenital heart disease (CCHD) was assessed before discharge from maternity hospital.Basic information was provided for screening CCHD in the early neonatal stage.Chi-squared test was used for comparison of categorical variables(detection rate of different types of CCHD). Method: A retrospective cohort study was conducted in neonates with CCHD who were admitted to Children's Hospital of Fudan University between 1 January 2012 and 31 December 2015. For comparing with the previously reported undiagnosed rate of CCHD at discharge, CCHD was defined as all duct dependent congenital heart disease (DDCHD) and any cyanotic CHD that required early surgery...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28441153/discharge-criteria-for-the-late-preterm-infant-a-review-of-the-literature
#12
Jenny M Quinn, Marteen Sparks, Sheila M Gephart
BACKGROUND: Standardized late preterm infant (LPI) discharge criteria ensure best practice and help guide the neonatal provider to determine the appropriate level of care following birth. However, the location can vary from the well newborn setting to the neonatal intensive care unit (NICU). PURPOSE: The purpose of this review is to examine differences in LPI discharge criteria between the well newborn setting and the NICU by answering the clinical questions, "What are the recommended discharge criteria for the LPI and do they differ if admitted to the well newborn setting versus the NICU?" SEARCH STRATEGY: Databases searched include CINAHL, TRIP, PubMed, and the Cochrane Library...
April 24, 2017: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
https://www.readbyqxmd.com/read/28434863/spatiotemporally-controlled-mechanical-cues-drive-progenitor-mesenchymal-to-epithelial-transition-enabling-proper-heart-formation-and-function
#13
Timothy R Jackson, Hye Young Kim, Uma L Balakrishnan, Carsten Stuckenholz, Lance A Davidson
During early cardiogenesis, bilateral fields of mesenchymal heart progenitor cells (HPCs) move from the anterior lateral plate mesoderm to the ventral midline, undergoing a mesenchymal-to-epithelial transition (MET) en route to forming a single epithelial sheet. Through tracking of tissue-level deformations in the heart-forming region (HFR) as well as movement trajectories and traction generation of individual HPCs, we find that the onset of MET correlates with a peak in mechanical stress within the HFR and changes in HPC migratory behaviors...
May 8, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28422173/critical-involvement-of-zeb2-in-collagen-fibrillogenesis-the-molecular-similarity-between-mowat-wilson-syndrome-and-ehlers-danlos-syndrome
#14
Mika Teraishi, Mikiro Takaishi, Kimiko Nakajima, Mitsunori Ikeda, Yujiro Higashi, Shinji Shimoda, Yoshinobu Asada, Atsushi Hijikata, Osamu Ohara, Yoko Hiraki, Seiji Mizuno, Toshiyuki Fukada, Takahisa Furukawa, Nobuaki Wakamatsu, Shigetoshi Sano
Mowat-Wilson syndrome (MOWS) is a congenital disease caused by de novo heterozygous loss of function mutations or deletions of the ZEB2 gene. MOWS patients show multiple anomalies including intellectual disability, a distinctive facial appearance, microcephaly, congenital heart defects and Hirschsprung disease. However, the skin manifestation(s) of patients with MOWS has not been documented in detail. Here, we recognized that MOWS patients exhibit many Ehlers-Danlos syndrome (EDS)-like symptoms, such as skin hyperextensibility, atrophic scars and joint hypermobility...
April 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28377475/diagnosis-imaging-and-clinical-management-of-aortic-coarctation
#15
REVIEW
Elles J Dijkema, Tim Leiner, Heynric B Grotenhuis
Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of CoA remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (>20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV)...
April 4, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28371265/oral-facial-digital-syndrome-type-1-in-males-congenital-heart-defects-are-included-in-its-phenotypic-spectrum
#16
Arjan Bouman, Mariëlle Alders, Roelof Jan Oostra, Elisabeth van Leeuwen, Nikki Thuijs, Anne-Marie van der Kevie-Kersemaekers, Merel van Maarle
Oral-facial-digital syndrome type 1 (OFD1; OMIM# 311200) is an X-linked dominant ciliopathy caused by mutations in the OFD1 gene. This condition is characterized by facial anomalies and abnormalities of oral tissues, digits, brain, and kidneys. Almost all affected patients are female, as OFD1 is presumed to be lethal in males, mostly in the first or second trimester of pregnancy. Live born males with OFD1 are a rare occurrence, with only five reported patients to date. In four patients the presence of a congenital heart defect (CHD) was observed...
May 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28329463/current-practices-in-the-timing-of-stage-2-palliation
#17
James M Meza, Robert D B Jaquiss, Brett R Anderson, Michael-Alice Moga, James K Kirklin, George Sarris, William G Williams, Brian W McCrindle
BACKGROUND: Mortality through single-ventricle palliation remains high and the effect of the timing of stage 2 palliation (S2P) is not well understood. We investigated current practice patterns in the timing of S2P across two professional societies and compared them to actual practice patterns from two databases of patients who underwent S2P. METHODS: A ten-question survey was distributed to the members of the Congenital Heart Surgeons' Society (CHSS) and the European Congenital Heart Surgeons' Association (ECHSA)...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28329450/public-views-on-pulse-oximetry-screening-for-critical-congenital-heart-disease
#18
MULTICENTER STUDY
Praveen Kumar, Hari Iyengar, Prerna Kumar
OBJECTIVES: To understand public views on pulse oximetry screening for critical congenital heart disease. METHODS: Two hundred thirteen adults read a brief vignette describing the importance of early detection of critical congenital heart disease and then answered five questions on a five-point scale of how likely or unlikely they were to support pulse oximetry screening. Responses were tabulated and analyzed using a Fisher exact test, and logistic regression was used to estimate odds ratios for adjusted associations using generalized estimating equations...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28329105/medical-three-dimensional-printing-opens-up-new-opportunities-in-cardiology-and-cardiac-surgery
#19
Thomas Bartel, Andrew Rivard, Alejandro Jimenez, Carlos A Mestres, Silvana Müller
Advanced percutaneous and surgical procedures in structural and congenital heart disease require precise pre-procedural planning and continuous quality control. Although current imaging modalities and post-processing software assists with peri-procedural guidance, their capabilities for spatial conceptualization remain limited in two- and three-dimensional representations. In contrast, 3D printing offers not only improved visualization for procedural planning, but provides substantial information on the accuracy of surgical reconstruction and device implantations...
February 16, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28325567/microvascular-surgery-in-the-congenital-cardiac-patient-a-case-series-exploring-feasibility-and-practical-applications
#20
John A LoGiudice, Karri Adamson, Nancy Ghanayem, Ronald K Woods, Michael E Mitchell
BACKGROUND: Pediatric congenital heart disease patients are at risk for vascular injuries during surgical procedures or when the arterial system is accessed for monitoring or diagnostic studies. Our treatment of emergent situations in this patient population using microvascular techniques shows the feasibility of such techniques. METHODS: A retrospective chart review of patients aged 0-18 years with congenital heart disease identified six patients who underwent microvascular surgery by the senior surgeon from June 2007 to May 2015...
May 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
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