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https://www.readbyqxmd.com/read/28993849/identification-of-novel-congenital-heart-disease-candidate-genes-using-chromosome-microarray
#1
Enas Shanshen, Janine Rosenberg, Andrew H Van Bergen
While the majority of patients have isolated heart disease, congenital heart disease (CHD) may be associated with other congenital anomalies or syndromes. Our institution utilizes chromosomal microarray (CMA) to identify chromosomal abnormalities, specifically copy number variations (CNVs). While CNVs have been associated with CHD, their direct impact on cardiac development remains unclear. This study sought to identify potential novel CHD candidate genes by comparing CNVs present in our institution's CHD population with those already recognized in the literature...
October 9, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28980093/incidence-of-fever-and-positive-bacterial-cultures-in-neonates-receiving-prostaglandin
#2
Fares Alghanem, Stephanie L Rakestraw, Kurt R Schumacher, Gabe E Owens
Prostaglandin E (PGE1) is necessary to maintain ductus arteriosus patency in many newborns with congenital heart disease. Because PGE1 therapy commonly leads to fever, and given this population's fragile state, a complete sepsis workup is often performed in febrile, but otherwise asymptomatic, patients. This practice of liberal evaluation with bacterial cultures, empiric antibiotic treatment, and delays in essential surgical intervention may result in poor resource utilization and lead to increased iatrogenic morbidity...
October 4, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28980091/biopsychosocial-predictors-of-quality-of-life-outcomes-in-pediatric-congenital-heart-disease
#3
Michelle M Ernst, Bradley S Marino, Amy Cassedy, Carrie Piazza-Waggoner, Rodney C Franklin, Katherine Brown, Jo Wray
The purpose of this prospective multi-center cross-sectional study was to identify key biopsychosocial factors that impact quality of life (QOL) of youth with congenital heart disease (CHD). Patient-parent pairs were recruited at a regular hospital follow-up visit. Patient- and parent-proxy-reported QOL were assessed using the Pediatric Cardiac Quality of Life Inventory (PCQLI). Wallander's and Varni's disability-stress coping model guided factor selection, which included disease factors, educational impairment, psychosocial stress, child psychological and parent/family factors...
October 4, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28970652/the-need-for-extracorporeal-membrane-oxygenation-in-adults-undergoing-congenital-heart-surgery-impact-and-trends-of-utilization
#4
Salvatore Aiello, Rohit S Loomba, Connor Kriz, Matthew Buelow, Saurabh Aggarwal, Rohit R Arora
INTRODUCTION: Adults with congenital heart disease (ACHD) represent a population with unique health-care needs. Many patients require cardiac surgery, with some requiring postoperative extracorporeal membrane oxygenation (ECMO). This study aimed to identify the risk factors for the need of postoperative ECMO and characterize the impact of ECMO on admission characteristics. METHODS: Data from the 2005-2012 iterations of the Nationwide Inpatient Sample were used. ACHD admissions over 18 years with a documented cardiac surgery were included...
September 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28970037/a-novel-method-for-evaluating-regional-rv-function-in-the-adult-congenital-heart-with-low-dose-ct-and-squeez-processing
#5
Francisco J Contijoch, Daniel W Groves, Zhennong Chen, Marcus Y Chen, Elliot R McVeigh
BACKGROUND: Measuring local RV function in adult congenital heart disease (ACHD) with echocardiography or MRI is challenging because of the complex geometry and existing pacing devices. Visual assessment of ventricular function via low-dose cardiac CT has been recently performed. This pilot study assessed whether low-dose 4D cine CT combined with automatic measurement of regional shortening could quantify right-ventricular function in ACHD patients. METHODS: Seven patients with Tetralogy of Fallot either contraindicated for MRI or assessed for coronary artery disease and seven non-congenital patients were imaged with ECG-gated cardiac CT utilizing a 320-detector row scanner...
September 29, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28948646/a-practical-guide-to-cardiovascular-3d-printing-in-clinical-practice-overview-and-examples
#6
Islam Abudayyeh, Brent Gordon, Mohammad M Ansari, Kenneth Jutzy, Liset Stoletniy, Anthony Hilliard
The advent of more advanced 3D image processing, reconstruction, and a variety of three-dimensional (3D) printing technologies using different materials has made rapid and fairly affordable anatomically accurate models much more achievable. These models show great promise in facilitating procedural and surgical planning for complex congenital and structural heart disease. Refinements in 3D printing technology lend itself to advanced applications in the fields of bio-printing, hemodynamic modeling, and implantable devices...
September 25, 2017: Journal of Interventional Cardiology
https://www.readbyqxmd.com/read/28942735/representation-of-cardiovascular-magnetic-resonance-in-the-aha-acc-guidelines
#7
Florian von Knobelsdorff-Brenkenhoff, Guenter Pilz, Jeanette Schulz-Menger
BACKGROUND: Whereas evidence supporting the diagnostic value of cardiovascular magnetic resonance (CMR) has increased, there exists significant worldwide variability in the clinical utilization of CMR. A recent study demonstrated that CMR is represented in the majority of European Society for Cardiology (ESC) guidelines, with a large number of specific recommendations in particular regarding coronary artery disease. To further investigate the gap between the evidence and clinical use of CMR, this study analyzed the role of CMR in the guidelines of the American College of Cardiology (ACC) and American Heart Association (AHA)...
September 25, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28932552/chylothorax-after-pediatric-cardiac-surgery-complicates-short-term-but-not-long-term-outcomes-a-propensity-matched-analysis
#8
Nikoletta R Czobor, György Roth, Zsolt Prodán, Daniel J Lex, Erzsébet Sápi, László Ablonczy, Mihály Gergely, Edgar A Székely, János Gál, Andrea Székely
BACKGROUND: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery. METHODS: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28924118/atrial-arrhythmia-ablation-in-adult-congenital-heart-disease-with-a-persistent-left-side-superior-vena-cava
#9
Shinya Yamada, Li-Wei Lo, Yenn-Jiang Lin, Atul Prabhu, Shih-Ann Chen
We herein report a case of atrial tachycardia (AT) originating from the dilated coronary sinus (CS) connected to a persistent left superior vena cava. The earliest activation site of AT was localized at the superior CS ostium, identified using a novel ultra-rapid high-density mapping system (Rhythmia™). Successful ablation was performed at the corresponding position. This report demonstrated the utility of an ultra-rapid high-density mapping system in identifying arrhythmogenic foci in adult cases of congenital heart disease...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28914109/stem-cell-therapy-for-the-systemic-right-ventricle
#10
Ming-Sing Si, Richard G Ohye
In specific forms of congenital heart defects and pulmonary hypertension, the right ventricle (RV) is exposed to systemic levels of pressure overload. The RV is prone to failure in these patients because of its vulnerability to chronic pressure overload. As patients with a systemic RV reach adulthood, an emerging epidemic of RV failure has become evident. Medical therapies proven for LV failure are ineffective in treating RV failure. Areas covered: In this review, the pathophysiology of the failing RV under pressure overload is discussed, with specific emphasis on the pivotal roles of angiogenesis and oxidative stress...
November 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28901228/the-world-database-for-pediatric-and-congenital-heart-surgery-the-dawn-of-a-new-era-of-global-communication-and-quality-improvement-in-congenital-heart-disease
#11
REVIEW
James D St Louis, Hiromi Kurosawa, Richard A Jonas, Nestor Sandoval, Jorge Cervantes, Christo I Tchervenkov, Jeffery P Jacobs, Kisaburo Sakamoto, Giovanni Stellin, James K Kirklin
The World Society for Pediatric and Congenital Heart Surgery was founded with the mission to "promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service." Early on, the Society's members realized that a crucial step in meeting this goal was to establish a global database that would collect vital information, allowing cardiac surgical centers worldwide to benchmark their outcomes and improve the quality of congenital heart disease care...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28886170/homoarginine-a-prognostic-indicator-in-adolescents-and-adults-with-complex-congenital-heart-disease
#12
Tanja Raedle-Hurst, Marieke Mueller, Andreas Meinitzer, Winfried Maerz, Thomas Dschietzig
BACKGROUND: Homoarginine (hArg) has been shown to be of prognostic value in patients with chronic left heart failure. The present study aims to assess the clinical utility and prognostic value of hArg levels in patients with complex congenital heart disease (CHD). METHODS: Plasma hArg levels were measured in 143 patients with complex CHD and compared to clinical status, echocardiographic and laboratory parameters as well as the occurrence of adverse cardiac events...
2017: PloS One
https://www.readbyqxmd.com/read/28871362/electrocardiographic-markers-of-appropriate-implantable-cardioverter-defibrillator-therapy-in-young-people-with-congenital-heart-diseases
#13
Dunia Bárbara Benítez Ramos, Michel Cabrera Ortega, Jesús Castro Hevia, Margarita Dorantes Sánchez, Ailema Amelia Alemán Fernández, Osmin Castañeda Chirino, Marlenis Cruz Cardentey, Frank Martínez López, Roylan Falcón Rodríguez
Implantable cardioverter-defibrillators (ICDs) are increasingly utilized in patients with congenital heart disease (CHD). Prediction of the occurrence of shocks is important if improved patient selection is desired. The electrocardiogram (ECG) has been the first-line tool predicting the risk of sudden death, but data in CHD patients are lacking. We aim to evaluate the predictive value of electrocardiographic markers of appropriate therapy of ICD in young people with CHD. We conducted a prospective, longitudinal study, in twenty-six CHD patients (mean age 24...
September 4, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28866869/total-anomalous-pulmonary-venous-connection-tapvc-a%C3%A2-familial-cluster-of-3-siblings
#14
Jennifer M Acevedo, Simon Lee, Nina Gotteiner, Amy S Lay, Angira Patel
Total anomalous pulmonary venous connection (TAPVC) is a rare form of cyanotic congenital heart disease (CHD) that occurs when the pulmonary veins drain into a site other than the morphologic left atrium. As with other forms of CHD, TAPVC has been shown to cluster in families and is known to have genetic association. We report on a case series of familial TAPVC in three consecutive siblings. A combination of fetal echocardiography, transthoracic echocardiography, as well as cross-sectional imaging was utilized in the diagnosis as well as management of each sibling...
September 3, 2017: Echocardiography
https://www.readbyqxmd.com/read/28864597/parvovirus-b19-infection-in-children-with-arterial-ischemic-stroke
#15
MULTICENTER STUDY
Heather J Fullerton, Jorge M Luna, Max Wintermark, Nancy K Hills, Rafal Tokarz, Ying Li, Carol Glaser, Gabrielle A DeVeber, W Ian Lipkin, Mitchell S V Elkind
BACKGROUND AND PURPOSE: Case-control studies suggest that acute infection transiently increases the risk of childhood arterial ischemic stroke. We hypothesized that an unbiased pathogen discovery approach utilizing MassTag-polymerase chain reaction would identify pathogens in the blood of childhood arterial ischemic stroke cases. METHODS: The multicenter international VIPS study (Vascular Effects of Infection in Pediatric Stroke) enrolled arterial ischemic stroke cases, and stroke-free controls, aged 29 days through 18 years...
October 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28858090/prenatal-ultrasonic-diagnosis-of-absent-pulmonary-valve-syndrome-a-case-report
#16
Wen-Jun Zhang, Zhong-Lei Zhang, Jun-Jie Chang, Xiao-Yu Song
RATIONALE: Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease that is often associated with tetralogy of Fallot (TOF). Here, we report 2 cases of APVS associated with TOF diagnosed via fetal echocardiography and discuss their specific ultrasonographic characteristics. PATIENT CONCERNS: Two pregnant women with suspicion of fetal heart anomaly were referred from their local hospitals to our hospital for fetal malformation screening and detailed fetal echocardiography...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28836088/feasibility-assessment-for-successfully-visualizing-the-fetal-heart-utilizing-spatiotemporal-image-correlation
#17
Eisuke Inubashiri, Sayuri Tatedo, Naomi Nishiyama, Hiina Minami, Yukio Watanabe, Noriyuki Akutagawa, Katumaru Kuroki, Masaki Sugawara, Nobuhiko Maeda, Keiji Haseyama
PURPOSE: Spatiotemporal image correlation (STIC) is an excellent imaging modality for observing the fetal heart. High-quality STIC volume data are needed for an antenatal anatomic survey to diagnose congenital heart disease. We aimed to clarify the causes of unsuccessful STIC volume data acquisition and describe a more accurate, efficient STIC examination. METHODS: This cross-sectional study of 1124 women with fetuses assessed risk factors for unsuccessful acquisition of STIC volume data...
August 23, 2017: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/28828392/thermal-stability-as-a-determinant-of-aav-serotype-identity
#18
Antonette Bennett, Saajan Patel, Mario Mietzsch, Ariana Jose, Bridget Lins-Austin, Jennifer C Yu, Brian Bothner, Robert McKenna, Mavis Agbandje-McKenna
Currently, there are over 150 ongoing clinical trials utilizing adeno-associated viruses (AAVs) to target various genetic diseases, including hemophilia (AAV2 and AAV8), congenital heart failure (AAV1 and AAV6), cystic fibrosis (AAV2), rheumatoid arthritis (AAV2), and Batten disease (AAVrh.10). Prior to patient administration, AAV vectors must have their serotype, concentration, purity, and stability confirmed. Here, we report the application of differential scanning fluorimetry (DSF) as a good manufacturing practice (GMP) capable of determining the melting temperature (Tm) for AAV serotype identification...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28807423/coping-with-the-stress-in-the-cardiac-intensive-care-unit-can-mindfulness-be-the-answer
#19
Nadya Golfenshtein, Janet A Deatrick, Amy J Lisanty, Barbara Medoff-Cooper
BACKGROUND: Mothers of infants with complex congenital heart disease are exposed to increased stress which has been associated with numerous adverse health outcomes. The coping mechanisms these mothers use critically effect the familial illness adaptation and most likely infant outcomes. Currently no data-based strategies have been developed for mothers to facilitate their coping, and proactively promote their adaptation in the critical care settings. A potential strategy is mindfulness which is currently used in other clinical settings with stress-reduction effects...
August 11, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28781147/durable-mechanical-circulatory-support-in-teenagers-and-adults-with-congenital-heart-disease-a-systematic-review
#20
Jill M Steiner, Eric V Krieger, Karen K Stout, April Stempien-Otero, Claudius Mahr, Nahush A Mokadam, Joshua L Hermsen
BACKGROUND: Heart failure is the leading cause of morbidity and mortality for adults with congenital heart disease (ACHD). Many patients are ineligible for transplantation, and those who are eligible often face long wait times with high wait-list morbidity. Durable mechanical circulatory support (MCS) may be an option for many patients. This systematic review evaluates the published literature on the use of durable MCS in teenagers and adults with congenital heart disease. METHODS: A comprehensive search of MEDLINE (PubMed), EMBASE, and the Cochrane Library was performed electronically in July 2015 and updated in March 2016, guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines...
August 2, 2017: International Journal of Cardiology
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