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https://www.readbyqxmd.com/read/28224467/appropriate-use-of-genetic-testing-in-congenital-heart-disease-patients
#1
REVIEW
Seiji Ito, Kimberly A Chapman, Monisha Kisling, Anitha S John
PURPOSE OF REVIEW: Congenital heart disease (CHD) remains the most common birth defect, occurring in 1% of all births. Although the exact etiology of CHD is still largely unknown, it is thought to be an interaction of genetic and non-genetic factors. The purposes of this review are to summarize recent advances in CHD genetics and testing and to present a suggested algorithm for appropriate use of genetic testing in patients with CHD. RECENT FINDINGS: Advances in genetic testing technology are rapidly expanding the options for screening and are providing further insights into the genetic and molecular background of non-syndromic CHD...
March 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28211263/increased-regurgitant-flow-causes-endocardial-cushion-defects-in-an-avian-embryonic-model-of-congenital-heart-disease
#2
Stephanie M Ford, Matthew T McPheeters, Yves T Wang, Pei Ma, Shi Gu, James Strainic, Christopher Snyder, Andrew M Rollins, Michiko Watanabe, Michael W Jenkins
BACKGROUND: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart...
February 17, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28191656/utility-and-safety-of-combined-interventional-catheterization-and-electrophysiology-procedures-in-a-children-s-hospital
#3
S Yukiko Asaki, Jeffrey W Orcutt, Christina Y Miyake, Henri Justino, Caridad M DE LA Uz, Jeffrey J Kim, Santiago O Valdes, Athar M Qureshi
BACKGROUND: Interventional cardiac catheterization (cath) and electrophysiology (EP) procedures are not routinely performed together. There are several perceived barriers affecting this practice, though there are also advantages for both the patient and practitioner to a combined approach. METHODS: This was a single center retrospective study reviewing combined cath and EP procedures with a pre-procedural intention to intervene at Texas Children's Hospital from 2001 to 2014...
February 13, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28134395/ultrasound-examination-of-the-fetal-heart
#4
Katherine C Bishop, Jeffrey A Kuller, Brita K Boyd, Eleanor H Rhee, Stephen Miller, Piers Barker
Congenital heart disease (CHD) occurs in 4-13 per 1000 births in the United States. While many risk factors for CHD have been identified, more than 90% of cases occur in low-risk patients. Guidelines for fetal cardiac screening during the second trimester anatomy ultrasound have been developed by the International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) in order to improve antenatal detection rates and to standardize the fetal cardiac screening examination. Patients found to be at increased risk of CHD because of risk factors or an abnormal screening examination should be referred for second trimester fetal echocardiography...
January 2017: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/28091832/utility-and-safety-of-the-safesept%C3%A2-transseptal-guidewire-for-electrophysiology-studies-with-catheter-ablation-in-pediatric-and-congenital-heart-disease
#5
Joseph J Knadler, Jeffrey B Anderson, Ahmad S Chaouki, Richard J Czosek, Chad Connor, Timothy K Knilans, David S Spar
PURPOSE: The atrial transseptal procedure is used in catheter ablation of left-sided arrhythmias. Studies in adult patients have shown the SafeSept™ transseptal guidewire (SSTG) to be effective in atrial transseptal procedures. We analyzed our 5-year experience with SSTG use in pediatric and congenital heart disease patients undergoing catheter ablation. METHODS: This is a single-center retrospective analysis of patients undergoing catheter ablation from 2009 to 2014...
January 14, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
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
#6
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/28035425/myocardial-stress-perfusion-magnetic-resonance-initial-experience-in-a-pediatric-and-young-adult-population-using-regadenoson
#7
Cory V Noel, Ramkumar Krishnamurthy, Brady Moffett, Rajesh Krishnamurthy
BACKGROUND: Dipyridamole and adenosine are traditional pharmacological stressors for myocardial perfusion. Regadenoson, a selective adenosine A2A agonist, has a lower side effect profile with lower incidence of bronchospasm and bradycardia. There is a growing need for myocardial perfusion assessment within pediatrics. There is no report on the utility of regadenoson as a stress agent in children. OBJECTIVE: To observe the safety and feasibility of regadenoson as a pharmacologic stressor for perfusion cardiac MR in a pilot cohort of pediatric patients weighing more than 40 kg who have congenital heart disease and pediatric acquired heart disease...
December 29, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/28033081/clinical-databases-and-registries-in-congenital-and-pediatric-cardiac-surgery-cardiology-critical-care-and-anesthesiology-worldwide
#8
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
https://www.readbyqxmd.com/read/28007066/valve-sparing-aortic-root-replacement-in-pediatric-patients-lessons-learned-over-two-decades
#9
REVIEW
Luca A Vricella, Duke E Cameron
Valve-sparing aortic root replacement has been utilized in children with aneurysms of the proximal aorta as a valid alternative to composite mechanical or biologic prostheses. While the majority of pediatric patients with aortic root aneurysms have various forms of connective tissue disorders, other diagnostic groups have emerged, such as patients with various forms of congenital heart disease and non-syndromic thoracic aortic aneurysms. We present our experience with valve-sparing procedures in pediatric patients, highlighting the technical aspects of the operation as well as some of the pitfalls and complications we have encountered over the last two decades...
January 2017: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/27956203/syphilis-during-pregnancy-a-preventable-threat-to-maternal-fetal-health
#10
REVIEW
Martha W F Rac, Paula A Revell, Catherine S Eppes
Syphilis remains the most common congenital infection worldwide and has tremendous consequences for the mother and her developing fetus if left untreated. Recently, there has been an increase in the number of congenital syphilis cases in the United States. Thus, recognition and appropriate treatment of reproductive-age women must be a priority. Testing should be performed at initiation of prenatal care and twice during the third trimester in high-risk patients. There are 2 diagnostic algorithms available and physicians should be aware of which algorithm is utilized by their testing laboratory...
December 9, 2016: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27894758/the-impact-of-cardiac-risk-factors-on-short-term-outcomes-for-children-undergoing-a-ladd-procedure
#11
Luke R Putnam, Kathryn T Anderson, KuoJen Tsao, Lillian S Kao, Jane A Lugo, Kevin P Lally, Akemi L Kawaguchi
BACKGROUND/PURPOSE: The purpose of this study was to describe the outcomes of children with and without congenital heart disease who undergo a Ladd procedure. METHODS: The 2012-2014 National Surgical Quality Improvement Program Pediatric (NSQIP-P) data were queried for patients undergoing a Ladd procedure. Utilizing NSQIP-P definitions, patients were categorized into four cardiac risk groups (none, minor, major, severe) based on severity of cardiac anomalies, previous cardiac procedure(s), and ongoing cardiac dysfunction...
October 21, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27883221/alcohol-exposure-causes-overexpression-of-heart-development-related-genes-by-affecting-the-histone-h3-acetylation-via-bmp-signaling-pathway-in-cardiomyoblast-cells
#12
Jin Shi, Weian Zhao, Bo Pan, Min Zheng, Lina Si, Jing Zhu, Lingjuan Liu, Jie Tian
BACKGROUND: Abusive alcohol utilization of pregnant woman may cause congenital heart disease (CHD) of fetus, where alcohol ignites histone H3 hyperacetylation leading to abnormal development of heart morphogenesis and associated genes. Knowledge about the regularized upstream genes is little, but bone morphogenetic protein (BMP) signaling may actively and prominently take part in alteration in acetylation of histone H3. The supreme objective of this study was to unearth the involvement of BMP signaling pathway in alcohol-driven hyperacetylation of histone H3 in cardiomyoblast cells...
January 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/27871578/children-with-heart-disease-risk-stratification-for-non-cardiac-surgery
#13
Angela K Saettele, Jacob L Christensen, Kelly L Chilson, David J Murray
STUDY OBJECTIVE: Children with congenital or acquired heart disease have an increased risk of anesthesia related morbidity and mortality. The child's anesthetic risk is related to the severity of their underlying cardiac disease, associated comorbidities, and surgical procedure. The goal of this project was to determine the ease of use of a preoperative risk stratification tool for assigning pediatric cardiac staff and to determine the relative frequency that children with low, moderate, and high risk cardiac disease present for non-cardiac surgery at a tertiary pediatric hospital...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27866675/hemodynamics-of-patient-specific-aorta-pulmonary-shunt-configurations
#14
Senol Piskin, H Firat Altin, Okan Yildiz, Ihsan Bakir, Kerem Pekkan
Optimal hemodynamics in aorta-pulmonary shunt reconstruction is essential for improved post-operative recovery of the newborn congenital heart disease patient. However, prior to in vivo execution, the prediction of post-operative hemodynamics is extremely challenging due to the interplay of multiple confounding physiological factors. It is hypothesized that the post-operative performance of the surgical shunt can be predicted through computational blood flow simulations that consider patient size, shunt configuration, cardiac output and the complex three-dimensional disease anatomy...
January 4, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/27865183/feasibility-of-low-radiation-dose-retrospectively-gated-cardiac-ct-for-functional-analysis-in-adult-congenital-heart-disease
#15
Daniel W Groves, Laura J Olivieri, Sujata M Shanbhag, Kathie C Bronson, Jeannie H Yu, Evan A Nelson, Shirley F Rollison, Michael S Stagliano, Anitha S John, Karen Kuehl, Marcus Y Chen
BACKGROUND: The use of cardiac computed tomography (CT) in the evaluation of adult congenital heart disease patients is limited due to concerns of high radiation doses. The purpose of this study was to prospectively assess whether low radiation dose cardiac CT is feasible to evaluate ventricular systolic function in adults with congenital heart disease. METHODS: The study group included 30 consecutive patients with significant congenital heart disease who underwent a total of 35 ECG-gated cardiac CT scans utilizing a 320-detector row CT scanner...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27862979/cerebral-tissue-oxygenation-index-and-lactate-at-24-hours-postoperative-predict-survival-and-neurodevelopmental-outcome-after-neonatal-cardiac-surgery
#16
Safwat A Aly, David Zurakowski, Penny Glass, Kami Skurow-Todd, Richard A Jonas, Mary T Donofrio
IMPORTANCE: There are no well-established noninvasive biomarkers for identifying patients at risk for poor outcome after surgery for congenital heart disease. Few studies have assessed prognostic accuracy of cerebral tissue oxygenation index (cTOI) measured by near infrared spectroscopy (NIRS). OBJECTIVE: To assess the utility of noninvasive NIRS monitoring as a predictor of outcomes after neonatal cardiac surgery through measurement of cTOI. To examine the utility of noninvasive NIRS monitoring in combination with lactate concentration and inotropic score in prediction of outcomes after neonatal cardiac surgery...
November 10, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27860542/successful-fetal-tele-echo-at-a-small-regional-hospital
#17
Julianna Brown, Brian Holland
BACKGROUND: Prenatal diagnosis of complex congenital heart disease (CHD) has been shown to improve newborn outcomes. The rate of prenatal diagnosis and access to fetal echocardiography vary widely across the United States. INTRODUCTION: A clinical fetal tele-echo service was established at King's Daughters Medical Center (KDMC) in Ashland, KY, a region in eastern Kentucky that is 3 h from the nearest congenital heart surgeon. The aim of this study was to determine if fetal tele-echo utilizing local sonographers at a small regional hospital can accurately and efficiently identify fetuses with complex CHD...
November 18, 2016: Telemedicine Journal and E-health: the Official Journal of the American Telemedicine Association
https://www.readbyqxmd.com/read/27855985/can-time-averaged-flow-boundary-conditions-be-used-to-meet-the-clinical-timeline-for-fontan-surgical-planning
#18
Zhenglun Alan Wei, Phillip M Trusty, Mike Tree, Christopher M Haggerty, Elaine Tang, Mark Fogel, Ajit P Yoganathan
Cardiovascular simulations have great potential as a clinical tool for planning and evaluating patient-specific treatment strategies for those suffering from congenital heart diseases, specifically Fontan patients. However, several bottlenecks have delayed wider deployment of the simulations for clinical use; the main obstacle is simulation cost. Currently, time-averaged clinical flow measurements are utilized as numerical boundary conditions (BCs) in order to reduce the computational power and time needed to offer surgical planning within a clinical time frame...
January 4, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/27837304/utility-and-scope-of-rapid-prototyping-in-patients-with-complex-muscular-ventricular-septal-defects-or-double-outlet-right-ventricle-does-it-alter-management-decisions
#19
Puneet Bhatla, Justin T Tretter, Achi Ludomirsky, Michael Argilla, Larry A Latson, Sujata Chakravarti, Piers C Barker, Shi-Joon Yoo, Doff B McElhinney, Nicole Wake, Ralph S Mosca
Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV)...
January 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/27801690/five-decades-of-pediatric-heart-transplantation-challenges-overcome-challenges-remaining
#20
Steven Zangwill
PURPOSE OF REVIEW: Pediatric heart transplants continue to be the therapy of choice for children with end stage heart failure. The interplay of limited donor supply, improvement in ventricular assist device (VAD) technology and utilization, and a focus on optimizing long-term outcomes make it critically important for practitioners to be aware of an evolving diagnostic and therapeutic arsenal. RECENT FINDINGS: Data suitable to define best practices for pediatric heart transplantation consist of an amalgam of small single center series, registry reviews and judicious inference from adult studies...
January 2017: Current Opinion in Cardiology
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