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Congenital Heart Disease

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https://www.readbyqxmd.com/read/28523862/transatlantic-medical-consultation-and-second-opinion-in-pediatric-cardiology-has-benefit-past-patient-care-a-case-study-in-videoconferencing
#1
Lubica Kovacikova, Martin Zahorec, Peter Skrak, Brian D Hanna, R Lee Vogel
BACKGROUND: Telemedicine is a rapidly evolving form of modern information and communication technology used to deliver clinical services and educational activities. OBJECTIVE: The aim of this article is to report and analyze our experience with transatlantic consultation via videoconferencing in pediatric cardiology. METHODS: In February, 2013, videoconferencing project was launched between a medium-volume pediatric cardiac center in Bratislava, Slovakia and subspecialty experts from a high-volume pediatric cardiac program at The Children's Hospital of Philadelphia (CHOP), USA...
May 19, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28523857/arrhythmia-burden-and-related-outcomes-in-eisenmenger-syndrome
#2
Shankar Baskar, Philippa Horne, Samantha Fitzsimmons, Philip R Khoury, Joseph Vettukattill, Koichiro Niwa, Teiji Agaki, Mark Spence, Hisanori Sakazaki, Gruschen Veldtman
BACKGROUND: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death. OBJECTIVE: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger's syndrome and to identify risk factors for arrhythmias. METHODS: This retrospective study included patients aged ≥ 18 years of age with Eisenmenger's syndrome from three institutions...
May 19, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28523852/preparing-adolescents-with-heart-problems-for-transition-to-adult-care-2009-2010-national-survey-of-children-with-special-health-care-needs
#3
Karrie F Downing, Matthew E Oster, Sherry L Farr
OBJECTIVE: A substantial percentage of children with congenital heart disease (CHD) fail to transfer to adult care, resulting in increased risk of morbidity and mortality. Transition planning discussions with a provider may increase rates of transfer, yet little is known about frequency and content of these discussions. We assessed prevalence and predictors of transition-related discussions between providers and parents of children with special healthcare needs (CSHCN) and heart problems, including CHD...
May 19, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28523732/long-term-respiratory-outcomes-after-primary-total-correction-for-tetralogy-of-fallot-and-absent-pulmonary-valve-in-patient-with-respiratory-symptoms
#4
Takaya Hoashi, Toru Iwasa, Koji Kagisaki, Masatoshi Shimada, Kenichi Kurosaki, Isao Shiraishi, Hajime Ichikawa
OBJECTIVE: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations. METHODS: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support...
May 19, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28508510/impaired-atrioventricular-transport-in-patients-with-transposition-of-the-great-arteries-palliated-by-atrial-switch-and-preserved-systolic-right-ventricular-function-a-magnetic-resonance-imaging-study
#5
Magalie Ladouceur, Nadjia Kachenoura, Gilles Soulat, Emilie Bollache, Alban Redheuil, Michel Azizi, Christophe Delclaux, Gilles Chatellier, Pierre Boutouyrie, Laurence Iserin, Damien Bonnet, Elie Mousseaux
OBJECTIVES: We aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship of these diastolic parameters with exercise performance and BNP, in patients with preserved systolic systemic right ventricle function. DESIGN: Single-center, cross-sectional, prospective study...
May 16, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28493451/the-effect-of-an-electronic-health-record-based-tool-on-abnormal-pediatric-blood-pressure-recognition
#6
Sarah A Twichell, Corinna J Rea, Patrice Melvin, Andrew J Capraro, Joshua C Mandel, Michael A Ferguson, Daniel J Nigrin, Kenneth D Mandl, Dionne Graham, Justin P Zachariah
BACKGROUND: Recognition of high blood pressure (BP) in children is poor, partly due to the need to compute age-sex-height referenced percentiles. This study examined the change in abnormal BP recognition before versus after the introduction of an electronic health record (EHR) app designed to calculate BP percentiles with a training lecture. METHODS AND RESULTS: Clinical data were extracted on all ambulatory, non-urgent encounters for children 3-18 years old seen in primary care, endocrinology, cardiology, or nephrology clinics at an urban, academic hospital in the year before and the year after app introduction...
May 11, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28493446/family-perception-of-unmet-support-needs-following-a-diagnosis-of-congenital-coronary-anomaly-in-children-results-of-a-survey
#7
Hitesh Agrawal, Oriana K Wright, Kathleen E Carberry, S Kristen Sexson Tejtel, Carlos M Mery, Silvana Molossi
BACKGROUND: Long-term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families. METHODS: An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed. RESULTS: A total of 31 (47%) families responded...
May 11, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28480627/predicting-long-term-mortality-after-fontan-procedures-a-risk-score-based-on-6707-patients-from-28-studies
#8
Tarek Alsaied, Jouke P Bokma, Mark E Engel, Joey M Kuijpers, Samuel P Hanke, Liesl Zuhlke, Bin Zhang, Gruschen R Veldtman
BACKGROUND: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting. METHODS: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported...
May 8, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28464509/forced-vital-capacity-predicts-morbidity-and-mortality-in-adults-with-repaired-tetralogy-of-fallot
#9
Katie E Cohen, Matthew W Buelow, Jennifer Dixon, Ruta Brazauskas, Scott B Cohen, Michael G Earing, Salil Ginde
OBJECTIVE: Abnormal lung function characterized by a reduced forced vital capacity (FVC) is common in adults with repaired tetralogy of Fallot (TOF) and is associated with previous thoracotomies and sternotomies. The impact of abnormal lung function on clinical outcomes in adult patients with repaired TOF is unclear. The aim of this study was to determine the impact of abnormal lung function on the outcome of hospitalization and death in adults with repaired TOF when analyzed with other traditional cardiac risk factors...
May 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28419713/nt-probnp-predicts-mortality-in-adults-with-transposition-of-the-great-arteries-late-after-mustard-or-senning-correction
#10
Jana Rubáčková Popelová, Markéta Tomková, Jakub Tomek
OBJECTIVE: The patients after Mustard and Senning corrections of transposition of the great arteries (TGA) are at an increased risk of unexpected death. The aim of this study was to identify markers allowing risk stratification of patients after atrial switch correction of TGA to provide them with optimum care. METHODS AND RESULTS: In this study, 87 patients were retrospectively evaluated after atrial switch correction of TGA followed-up between 2005 and 2015. The mortality during the follow-up was 9% (8 cardiac deaths)...
April 17, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28371362/pregnancy-related-acute-aortic-dissection-in-marfan-syndrome-a-review-of-the-literature
#11
REVIEW
Katherine Smith, Bernard Gros
A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified...
April 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28326674/whole-exome-sequencing-with-genomic-triangulation-implicates-cdh2-encoded-n-cadherin-as-a-novel-pathogenic-substrate-for-arrhythmogenic-cardiomyopathy
#12
Kari L Turkowski, David J Tester, J Martijn Bos, Kristina H Haugaa, Michael J Ackerman
BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is a heritable disease characterized by fibrofatty replacement of cardiomyocytes, has a prevalence of approximately 1 in 5000 individuals, and accounts for approximately 20% of sudden cardiac death in the young (≤35 years). ACM is most often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. While mutations in several genes that encode key desmosomal proteins underlie about half of all ACM, the remainder is elusive genetically...
March 21, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28299882/systolic-ejection-click-versus-split-first-heart-sound-are-our-ears-deceiving-us
#13
Natalie M Hoeting, Courtney E McCracken, Michael McConnell, Denver Sallee, Glen J Iannucci, Matthew E Oster
OBJECTIVE: Bicuspid aortic valve (BAV) disease is associated with potential lifetime complications, but auscultation of a BAV click is commonly missed or mistaken for a benign split first heart sound. Our objective was to determine whether pediatric cardiologists could reliably distinguish between BAV clicks and benign split first heart sounds. DESIGN: Quality evaluation project using de-identified recordings from an outpatient pediatric cardiology clinic. OUTCOME MEASURES: Twenty-one cardiologists listened to five de-identified recordings of pediatric heart sounds (three with BAV clicks, two with mitral components of benign split first heart sounds) and indicated whether they believed each recording was a BAV or split first heart sound...
March 16, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28299880/rates-of-autism-and-potential-risk-factors-in-children-with-congenital-heart-defects
#14
Jessica L Bean Jaworski, Thomas Flynn, Nancy Burnham, Jesse L Chittams, Therese Sammarco, Marsha Gerdes, Judy C Bernbaum, Robert R Clancy, Cynthia B Solot, Elaine H Zackai, Donna M McDonald-McGinn, J William Gaynor
OBJECTIVE: Atypical development, behavioral difficulties, and academic underachievement are common morbidities in children with a history of congenital heart defects and impact quality of life. Language and social-cognitive deficits have been described, which are associated with autism spectrum disorders. The current study aimed to assess the rates of autism spectrum disorders in a large sample of children with a history of congenital heart defects and to assess medical, behavioral, and individual factors that may be associated with the risk of autism spectrum disorders...
March 16, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28294539/angiographically-detectable-thebesian-veins-are-a-dynamic-and-reversible-finding-in-the-setting-of-congenital-heart-disease
#15
Paul Tannous, Sunil J Ghelani, Audrey C Marshall, Diego Porras
OBJECTIVE: Angiographically detectable Thebesian veins (ThVs) are a rare finding sometimes associated with coronary steal and myocardial ischemia in adults, but there are limited data regarding prominent ThVs in the setting of complex congenital heart disease (CHD). This study represents the largest series to date describing the presence and temporal changes of angiographically detectable ThVs in children with CHD. METHODS: This is a single center case series describing the clinical characteristics and coronary anatomy in children with CHD and angiographicall detectable ThVs...
March 10, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28244680/dysphagia-in-infants-with-single-ventricle-anatomy-following-stage-1-palliation-physiologic-correlates-and-response-to-treatment
#16
Katlyn Elizabeth McGrattan, Heather McGhee, Allan DeToma, Elizabeth G Hill, Sinai C Zyblewski, Maureen Lefton-Greif, Lucinda Halstead, Scott M Bradley, Bonnie Martin-Harris
BACKGROUND: Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood. OBJECTIVE: Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery...
February 28, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28240408/tilt-table-testing-to-diagnose-pseudosyncope-in-the-pediatric-population
#17
Jeffrey A Robinson, Jill K Shivapour, Christopher S Snyder
OBJECTIVE: Pseudosyncope can be difficult to distinguish from true syncope. Often, pediatric patients with pseudosyncope undergo multiple tests and referrals before the appropriate diagnosis is reached. The purpose is to describe the utility of the head-up tilt table test to elicit the diagnosis of pseudosyncope in the pediatric population. DESIGN: Retrospective chart review from November 2012 to December 2015 of patients age ≤23 years referred for 30-minute, 80-degree tilt table test...
February 27, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28382780/denton-cooley
#18
EDITORIAL
Douglas S Moodie
No abstract text is available yet for this article.
March 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28382779/peri-procedural-risk-stratification-and-management-of-patients-with-williams-syndrome
#19
REVIEW
R Thomas Collins Ii, Margaret G Collins, Michael L Schmitz, Justin T Hamrick
Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period. Detailed guidelines for peri-procedural management of patients with WS are limited...
March 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28140526/surveillance-for-liver-complications-after-the-fontan-procedure
#20
REVIEW
Moira B Hilscher, Jonathan N Johnson, Frank Cetta, David J Driscoll, John J Poterucha, William Sanchez, Heidi M Connolly, Patrick S Kamath
The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population...
March 2017: Congenital Heart Disease
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