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https://www.readbyqxmd.com/read/28230599/a-targeted-next-generation-genetic-sequencing-study-on-tetralogy-of-fallot-combined-with-cleft-lip-and-palate
#1
Lin Liu, Haisong Bu, Yifeng Yang, Zhiping Tan, Fei Zhang, Shijun Hu, Tianli Zhao
BACKGROUND: Congenital heart disease (CHD), plus cleft lip and palate (CLP) are currently the most common types of structural malformation in infants. Many genes have been investigated for their involvement in CHD with CLP. Targeted next-generation sequencing can analyze large amounts of genetic information rapidly, and thus address this question. METHODS: The authors designed a targeted, next-generation sequencing gene panel for 455 genes previously implicated in CHD or CLP...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28229912/mid-diastolic-left-ventricular-volume-and-mass-normal-values-for-coronary-computed-tomography-angiography
#2
Daniel Juneau, Fernanda Erthal, Owen Clarkin, Atif Alzahrani, Ali Alenazy, Alomgir Hossain, Joao R Inacio, Girish Dwivedi, Alexander J Dick, Frank J Rybicki, Benjamin J W Chow
BACKGROUND: The adoption of prospectively ECG-triggered acquisition coronary computed tomography angiography (CTA) has resulted in the inability to measure left ventricle (LV) end-diastolic volume and LV ejection fraction. However other prognostic measures such as LV mass and LV mid-diastolic volume (LVMDV) can still be assessed. The objective of this study is to establish normal reference values for LVMDV and LV mass. METHODS: Left ventricular mid-diastolic volumes and LV mass were prospectively measured in 2647 consecutive 'normal' patients undergoing prospectively ECG-triggered coronary CTA...
February 10, 2017: Journal of Cardiovascular Computed Tomography
https://www.readbyqxmd.com/read/28228731/increased-hemodynamic-load-in-early-embryonic-stages-alters-endocardial-to-mesenchymal-transition
#3
Madeline Midgett, Claudia S López, Larry David, Alina Maloyan, Sandra Rugonyi
Normal blood flow is essential for proper heart formation during embryonic development, as abnormal hemodynamic load (blood pressure and shear stress) results in cardiac defects seen in congenital heart disease. However, the progressive detrimental remodeling processes that relate altered blood flow to cardiac defects remain unclear. Endothelial-mesenchymal cell transition is one of the many complex developmental events involved in transforming the early embryonic outflow tract into the aorta, pulmonary trunk, interventricular septum, and semilunar valves...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28228450/academic-outcomes-in-children-with-congenital-heart-defects-a-population-based-cohort-study
#4
Matthew E Oster, Stephanie Watkins, Kevin D Hill, Jessica H Knight, Robert E Meyer
BACKGROUND: Most studies evaluating neurocognitive outcomes in children with congenital heart defects (CHD) have focused on high-risk patients or used specialized, resource-intensive testing. To determine the association of CHD with academic outcomes and compare outcomes according to the severity of CHD, we linked state educational records with a birth defects registry and birth certificates. METHODS AND RESULTS: We performed a retrospective cohort study using data from the North Carolina Birth Defects Monitoring Program, North Carolina Department of Public Instruction, and North Carolina Department of Health and Human Services vital records...
February 2017: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/28228299/maternal-hyperhomocysteinemia-and-congenital-heart-defects-a-prospective-case-control-study-in-indian-population
#5
Reyaz A Malik, Mohd R Lone, Asif Ahmed, Kaisar A Koul, Rahid R Malla
OBJECTIVE: Very few studies have been conducted in this part of world to identify relation between maternal serum homocysteine levels and congenital heart disease in their offsprings. With this perspective in mind, this study was carried out. METHODS: Fifty women were enrolled in this study. Thirty of these had delivered neonates who were diagnosed to have congenital heart diseases. These were treated as cases. Twenty of these women had delivered neonates who did not have any congenital heart diseases and were treated as controls...
January 2017: Indian Heart Journal
https://www.readbyqxmd.com/read/28226725/a-chicken-embryo-cardiac-outflow-tract-atlas-for-registering-changes-due-to-abnormal-blood-flow
#6
James P Carson, Monique Y Rennie, Michael Danilchik, Kent Thornburg, Sandra Rugonyi, James P Carson, Monique Y Rennie, Michael Danilchik, Kent Thornburg, Sandra Rugonyi, Kent Thornburg, Michael Danilchik, James P Carson, Monique Y Rennie, Sandra Rugonyi
Subdivision-based image registration has previously been applied to co-localize digital information extracted from rigid structures in biological specimens, such as the brain. Here, we describe and demonstrate the creation and application of a two-dimensional subdivision-based atlas representing a dynamic structure: the outflow tract of the developing chicken heart. The atlas is designed to segment three different anatomical layers of the outflow tract, and is demonstrated on the characterization of collagen XIV in both control and induced abnormal flow specimens...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28226723/improving-assessment-of-congenital-heart-disease-through-rapid-patient-specific-modeling
#7
Kathleen Gilbert, Genevieve Farrar, Brett Cowan, Avan Suinesiaputra, Christopher Occleshaw, Beau Pontre, James Perry, Sanjeet Hegde, Jeffrey Omens, Andrew McCulloch, Alistair Young, Kathleen Gilbert, Genevieve Farrar, Brett Cowan, Avan Suinesiaputra, Christopher Occleshaw, Beau Pontre, James Perry, Sanjeet Hegde, Jeffrey Omens, Andrew McCulloch, Alistair Young, James Perry, Genevieve Farrar, Avan Suinesiaputra, Brett Cowan, Kathleen Gilbert, Jeffrey Omens, Christopher Occleshaw, Sanjeet Hegde, Beau Pontre, Andrew McCulloch, Alistair Young
Congenital heart disease is the most common birth defect, with an incidence of 75 in every 1000 births. As a result of improved interventions, 90% of people with congenital heart disease now survive to adulthood. They must undergo regular imaging to assess their biventricular (left and right ventricular) function. Analysis of the images is problematic due to the large variety of shapes and complex geometry. In this paper we extend a biventricular modeling method to improve the analysis of MR images from congenital heart disease patients...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28224467/appropriate-use-of-genetic-testing-in-congenital-heart-disease-patients
#8
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/28223051/functional-status-and-quality-of-life-in-survivors-of-extracorporeal-membrane-oxygenation-after-the-norwood-operation
#9
Joshua M Friedland-Little, Karen Uzark, Sunkyung Yu, Ray Lowery, Ranjit Aiyagari, Jennifer C Hirsch-Romano
BACKGROUND: Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors. METHODS: We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood...
February 18, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28221991/detecting-clinically-meaningful-shape-clusters-in-medical-image-data-metrics-analysis-for-hierarchical-clustering-applied-to-healthy-and-pathological-aortic-arches
#10
Jan L Bruse, Maria A Zuluaga, Abbas Khushnood, Kristin McLeod, Hopewell N Ntsinjana, Tain-Yen Hsia, Maxime Sermesant, Xavier Pennec, Andrew M Taylor, Silvia Schievano
OBJECTIVE: Today's growing medical image databases call for novel processing tools to structure the bulk of data and extract clinically relevant information. Unsupervised hierarchical clustering may reveal clusters within anatomical shape data of patient populations as required for modern Precision Medicine strategies. Few studies have applied hierarchical clustering techniques to three-dimensional patient shape data and results depend heavily on the chosen clustering distance metrics and linkage functions...
February 16, 2017: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/28221263/use-of-model-based-iterative-reconstruction-to-improve-detection-of-congenital-cardiovascular-anomalies-in-infants-undergoing-free-breathing-computed-tomographic-angiography
#11
Seth Kligerman, Ferdia Bolster, Jason Mitchell, Travis Henry, Jean Jeudy, Charles S White
PURPOSE: The aim of the study was to assess the detection of congenital cardiovascular anomalies (congenital heart disease) in neonates and infants using model-based iterative reconstruction (MBIR) algorithm compared with hybrid iterative reconstruction (HIR) and filtered back projection (FBP) reconstructions on axial computed tomography (CT) performed at minimum scanner dose. MATERIALS AND METHODS: Over 1 year, all CT angiographies performed in infants below 3 months of age with congenital heart disease were assessed retrospectively...
March 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28219544/the-society-of-thoracic-surgeons-congenital-heart-surgery-database-2017-update-on-outcomes-and-quality
#12
Jeffrey P Jacobs, John E Mayer, Constantine Mavroudis, Sean M O'Brien, Erle H Austin, Sara K Pasquali, Kevin D Hill, David M Overman, James D St Louis, Tara Karamlou, Christian Pizarro, Jennifer C Hirsch-Romano, Donna McDonald, Jane M Han, Susan Becker, Christo I Tchervenkov, Francois Lacour-Gayet, Carl L Backer, Charles D Fraser, James S Tweddell, Martin J Elliott, Hal Walters, Richard A Jonas, Richard L Prager, David M Shahian, Marshall L Jacobs
The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28218213/surgical-treatment-of-double-outlet-right-ventricle-complicated-by-pulmonary-hypertension
#13
Qing-Yu Wu, Dong-Hai Li, Hong-Yin Li, Ming-Kui Zhang, Zhong-Hua Xu, Hui Xue
BACKGROUND: Double outlet right ventricle (DORV) is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH. METHODS: From June 2004 to November 2016, 61 patients (36 males and 25 females) aged 2 weeks to 26 years (median: 0.67 years and interquartile range: 0...
2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28217850/correlates-of-posttraumatic-stress-disorder-in-adults-with-congenital-heart-disease
#14
Bahareh Eslami
OBJECTIVE: The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics). DESIGN: Cross-sectional. SETTING: Two university-affiliated heart hospitals in Tehran, Iran. PATIENTS: A sample of 347 adults with congenital heart disease aged 18-64 years (52% women), and 353 adults without congenital heart disease matched by sex and age (±2 years) was recruited...
February 20, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28217064/a-simple-technique-to-achieve-vascular-access-for-continuous-venous-venous-ultrafiltration-in-a-toddler
#15
Joseph Drew Tobias
Acute renal failure is associated with increased mortality in the Pediatric Intensive Care Unit. When anuric or oliguric renal failure occurs, the associated fluid overload may compromise respiratory function and has been shown to be associated with worse outcomes. Renal replacement therapy using continuous venous-venous hemofiltration (CVVH) allows for fluid, solute, and nitrogenous waste removal. However, large bore vascular access with placement of a double-lumen dialysis catheter is necessary to ensure effective flow rates to allow for CVVH...
January 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28216495/-clinical-value-of-adma-in-patients-with-pulmonary-arterial-hypertension-combination-with-congenital-heart-disease
#16
Pengfei Chen, Yiyuan Huang, Zhenfei Fang, Xinqun Hu, Xiangqian Shen, Shenghua Zhou
To study the concentration of the asymmetrical dimethyl-L-arginine (ADMA) in patients with pulmonary arterial hypertension (PAH) commbination with congenital heart disease (CHD) and its clinical value as a biomaker for diagnosis and prognosis.
 Methods: A total of 100 patients with CHD and 25 healthy adult subjects were recruited. CHD patients were divided into three groups: normal pulmonary arterial pressure group (group A, n=25), mild-to-moderate PAH group (group B, n=25), severe PAH group (group C, n=50)...
January 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28215417/prenatal-diagnosis-of-the-criss-cross-heart
#17
Prasad Ravi, Deborah Fruitman, Lindsay Mills, Timothy Colen, Lisa K Hornberger
We sought to describe the prenatal features and postnatal outcomes of the "criss-cross heart" (CCH) with crossing atrioventricular connections as encountered in 5 cases and to review the literature regarding fetal diagnoses of this lesion. We reviewed the clinical history and fetal echocardiograms of 5 patients with a diagnosis of CCH encountered in our institution from 2010 to 2015. Affected pregnancies were identified through the University of Alberta fetal cardiology database. Fetal and postnatal echocardiograms, prenatal and postnatal clinical records, autopsy, and surgical reports were reviewed...
December 18, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/28215395/microarrays-in-prenatal-diagnosis
#18
REVIEW
Beatrice Oneda, Anita Rauch
In prenatal diagnosis, chromosomal microarray (CMA) has not yet fully replaced conventional karyotyping but has rapidly become the recommended test in pregnancies with ultrasound abnormalities. In this review, we provide an overview of the published data concerning this technology and the controversies concerning its use in the prenatal setting. There is abundant evidence indicating the added detection of pathogenic abnormalities with CMA in comparison to the traditional karyotyping, especially in fetuses with multiple or isolated ultrasound abnormalities such as congenital heart disease, increased nuchal translucency, or oral cleft...
January 23, 2017: Best Practice & Research. Clinical Obstetrics & Gynaecology
https://www.readbyqxmd.com/read/28214968/piloting-the-use-of-patient-specific-cardiac-models-as-a-novel-tool-to-facilitate-communication-during-cinical-consultations
#19
Giovanni Biglino, Despina Koniordou, Marisa Gasparini, Claudio Capelli, Lindsay-Kay Leaver, Sachin Khambadkone, Silvia Schievano, Andrew M Taylor, Jo Wray
This pilot study aimed to assess the impact of using patient-specific three-dimensional (3D) models of congenital heart disease (CHD) during consultations with adolescent patients. Adolescent CHD patients (n = 20, age 15-18 years, 15 male) were asked to complete two questionnaires during a cardiology transition clinic at a specialist centre. The first questionnaire was completed just before routine consultation with the cardiologist, the second just after the consultation. During the consultation, each patient was presented with a 3D full heart model realised from their medical imaging data...
February 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28214967/increased-atrial-%C3%AE-adrenergic-receptors-and-grk-2-gene-expression-can-play-a-fundamental-role-in-heart-failure-after-repair-of-congenital-heart-disease-with-cardiopulmonary-bypass
#20
Marcela Silva Oliveira, Fabio Carmona, Walter V A Vicente, Paulo H Manso, Karina M Mata, Mara Rúbia Celes, Erica C Campos, Simone G Ramos
Surgeries to correct congenital heart diseases are increasing in Brazil and worldwide. However, even with the advances in surgical techniques and perfusion, some cases, especially the more complex ones, can develop heart failure and death. A retrospective study of patients who underwent surgery for correction of congenital heart diseases with cardiopulmonary bypass (CPB) in a university tertiary-care hospital that died, showed infarction in different stages of evolution and scattered microcalcifications in the myocardium, even without coronary obstruction...
February 18, 2017: Pediatric Cardiology
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