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Congenital Heart Disease

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https://www.readbyqxmd.com/read/28805011/bicuspid-and-unicuspid-aortic-valves-different-phenotypes-of-the-same-disease-insight-from-the-gentac-registry
#1
Joseph M Krepp, Mary J Roman, Richard B Devereux, Adrienne Bruce, Siddharth K Prakash, Shaine A Morris, Dianna M Milewicz, Kathryn W Holmes, William Ravekes, Ralph V Shohet, Reed E Pyeritz, Cheryl L Maslen, Barbara L Kroner, Kim A Eagle, Liliana Preiss, Federico M Asch
BACKGROUND: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed. METHODS: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions...
August 14, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28795517/cardiovascular-adaptation-to-the-fontan-circulation
#2
REVIEW
Gruschen R Veldtman, Alexander R Opotowsky, Samuel G Wittekind, Jack Rychik, Daniel J Penny, Mark Fogel, Bradley S Marino, Marc Gewillig
Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations...
August 10, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28772057/consolidate-or-constipate-what-are-we-going-to-do-with-all-our-registries
#3
EDITORIAL
Andrew N Redington
No abstract text is available yet for this article.
August 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28762646/predicting-unplanned-readmissions-to-a-pediatric-cardiac-intensive-care-unit-using-predischarge-pediatric-early-warning-scores
#4
Ashley R Kroeger, Jacqueline Morrison, Andrew H Smith
OBJECTIVE: Unplanned readmission to the pediatric cardiac intensive care unit (CICU) is associated with significant morbidity and mortality. The Pediatric Early Warning Score (PEWS) predicts ward patients at risk for decompensation but has not been previously reported to identify at-risk patients with cardiac disease prior to ward transfer. This study aimed to determine whether PEWS prior to transfer may serve as a predictor of unplanned readmission to the CICU. DESIGN: All patients discharged from a tertiary children's hospital CICU from September 2012 through August 2015 were included for analysis...
August 1, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28744980/building-a-comprehensive-team-for-the-longitudinal-care-of-single-ventricle-heart-defects-building-blocks-and-initial-results
#5
Karen Texter, Jo Ann M Davis, Christina Phelps, Sharon Cheatham, John Cheatham, Mark Galantowicz, Timothy F Feltes
INTRODUCTION: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues. RESULTS: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution...
July 26, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28741863/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-infection-prevention
#6
Jonathan N Johnson, Cindy S Barrett, Wayne H Franklin, Eric M Graham, Nancy J Halnon, Brandy A Hattendorf, Catherine D Krawczeski, James J McGovern, Matthew J O'Connor, Amy H Schultz, Jeffrey M Vinocur, Devyani Chowdhury, Jeffrey B Anderson
INTRODUCTION: In 2012, the American College of Cardiology's (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee's metric design process...
July 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28737022/anatomic-types-of-anomalous-aortic-origin-of-a-coronary-artery-a-pictorial-summary
#7
Hitesh Agrawal, Carlos M Mery, Rajesh Krishnamurthy, Silvana Molossi
Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. As commonly seen in congenital heart disease, a wide spectrum of anatomic variability is seen and hence, it is important for clinicians to document the precise anatomy and course of the anomalous vessel. This article describes coronary artery nomenclature using computerized tomography angiography and virtual angioscopy. These details are important for decision making, useful for surgical planning, and may have prognostic implications...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28736987/cardiac-stress-mri-evaluation-of-anomalous-aortic-origin-of-a-coronary-artery
#8
Cory Noel
Myocardial ischemia is an insult that is primarily thought of in an adult population. However, there are several congenital and acquired cardiac lesions that may lead to myocardial ischemia in a pediatric population. One of the prominent congenital lesions is anomalous aortic origin of a coronary artery (AAOCA). Anomalous aortic origin of a coronary artery is one of the leading causes sudden cardiac death in pediatric and young adult patients, and thus the assessment of myocardial perfusion is of the utmost importance...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28736983/forgotten-knowledge-of-the-coronary-cameral-connections-and-the-rediscovery-of-their-dynamic-nature-in-development-the-result-of-a-misnomer-ambiguous-use-of-nomenclature-and-varied-evaluation-methods
#9
LETTER
https://www.readbyqxmd.com/read/28736841/outcomes-of-blalock-taussig-shunts-in-current-era-a-single-center-experience
#10
Navaneetha Sasikumar, Antony Hermuzi, Chun-Po Steve Fan, Kyong-Jin Lee, Rajiv Chaturvedi, Edward Hickey, Osami Honjo, Glen S Van Arsdell, Christopher A Caldarone, Arnav Agarwal, Lee Benson
OBJECTIVES: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity. DESIGN: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28736836/identification-of-adults-with-congenital-heart-disease-of-moderate-or-great-complexity-from-administrative-data
#11
Jill M Steiner, James N Kirkpatrick, Susan R Heckbert, Asma Habib, James Sibley, William Lober, J Randall Curtis
INTRODUCTION: There is relatively sparse literature on the use of administrative datasets for research in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the accuracy of administrative data for identifying patients with ACHD who died. METHODS: A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created. A search for these codes in the electronic health record of adults who received care in 2010-2016 was performed, and used state death records to identify patients who died during this period...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28736822/congenital-heart-disease-and-cardiac-procedural-outcomes-in-patients-with-trisomy-21-and-turner-syndrome
#12
Raysa Morales-Demori
Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%-60%), atrial (16%-21%), or ventricular septal defects (14%-27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#13
Constanze Pfitzer, Paul C Helm, Hannah Ferentzi, Lisa-Maria Rosenthal, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions)...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28719069/inaccuracy-of-a-continuous-arterial-pressure-waveform-monitor-when-used-for-congenital-cardiac-catheterization
#14
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/28719049/pulmonary-vein-stenosis-in-patients-with-smith-lemli-opitz-syndrome
#15
Aaron R Prosnitz, Jane Leopold, Mira Irons, Kathy Jenkins, Amy E Roberts
OBJECTIVE: To describe a group of children with co-incident pulmonary vein stenosis and Smith-Lemli-Opitz syndrome and to generate hypotheses as to the shared pathogenesis of these disorders. DESIGN: Retrospective case series. PATIENTS: Five subjects in a pulmonary vein stenosis cohort of 170 subjects were diagnosed with Smith-Lemli-Opitz syndrome soon after birth. RESULTS: All five cases were diagnosed with Smith-Lemli-Opitz syndrome within 6 weeks of life, with no family history of either disorder...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28691263/stenting-the-vertical-ductus-arteriosus-via-axillary-artery-access-using-wire-target-technique
#16
Tugcin Bora Polat
OBJECTIVES: To retrospectively review the outcome of stent placement in neonates with a vertical ductus, present a technique of ductal stenting via the axillary artery and compare it to ductal stening via the femoral venous access. DESIGN: Nineteen patients with duct-dependent pulmonary circulations through a vertical ductus arteriosus were treated with stent implantation. Those patients were retrospectively included in the study. In the first nine of these cases, stent delivery was done transvenously...
July 9, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28675696/myocardial-bridges-overview-of-diagnosis-and-management
#17
Ian S Rogers, Jennifer A Tremmel, Ingela Schnittger
A myocardial bridge is a segment of a coronary artery that travels into the myocardium instead of the normal epicardial course. Although it is general perception that myocardial bridges are normal variants, patients with myocardial bridges can present with symptoms, such as exertional chest pain, that cannot be explained by a secondary etiology. Such patients may benefit from individualized medical/surgical therapy. This article describes the prevalence, clinical presentation, classification, evaluation, and management of children and adults with symptomatic myocardial bridges...
July 3, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28670826/current-practices-are-variable-in-the-evaluation-and-management-of-patients-with-anomalous-aortic-origin-of-a-coronary-artery-results-of-a-survey
#18
Hitesh Agrawal, Carlos M Mery, Patrick E Day, S Kristen Sexson Tejtel, E Dean McKenzie, Charles D Fraser, Athar M Qureshi, Silvana Molossi
BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner. METHODS: An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA...
July 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28660735/impact-of-standardized-clinical-assessment-and-management-plans-on-resource-utilization-and-costs-in-children-after-the-arterial-switch-operation
#19
Rahul H Rathod, Brittney Jurgen, Rose A Hamershock, Kevin G Friedman, Audrey C Marshall, Mihail Samnaliev, Dionne A Graham, Kathy Jenkins, James E Lock, Andrew J Powell
BACKGROUND: Standardized Clinical Assessment and Management Plans (SCAMPs) are a quality improvement initiative designed to reduce unnecessary utilization, decrease practice variation, and improve patient outcomes. We created a novel methodology, the SCAMP managed episode of care (SMEOC), which encompasses multiple encounters to assess the impact of the arterial switch operation (ASO) SCAMP on total costs. METHODS: All ASO SCAMP patients (dates March 2009 to July 2015) were compared to a control group of ASO patients (January 2001 to February 2009)...
June 28, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28653469/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-chest-pain
#20
Jimmy C Lu, Manish Bansal, Sarina K Behera, Jeffrey R Boris, Brian Cardis, John S Hokanson, Bahram Kakavand, Roy Jedeikin
OBJECTIVE: As part of the American College of Cardiology Adult Congenital and Pediatric Cardiology Section effort to develop quality metrics (QMs) for ambulatory pediatric practice, the chest pain subcommittee aimed to develop QMs for evaluation of chest pain. DESIGN: A group of 8 pediatric cardiologists formulated candidate QMs in the areas of history, physical examination, and testing. Consensus candidate QMs were submitted to an expert panel for scoring by the RAND-UCLA modified Delphi process...
June 27, 2017: Congenital Heart Disease
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