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Congenital heart defect

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https://www.readbyqxmd.com/read/28237971/body-mass-index-in-adult-congenital-heart-disease
#1
Margarita Brida, Konstantinos Dimopoulos, Alexander Kempny, Emmanouil Liodakis, Rafael Alonso-Gonzalez, Lorna Swan, Anselm Uebing, Helmut Baumgartner, Michael A Gatzoulis, Gerhard-Paul Diller
OBJECTIVE: Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population. METHODS: We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (<18...
February 25, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28237970/clinical-predictors-of-length-of-stay-in-adults-with-congenital-heart-disease
#2
Ari Cedars, Lawrence Benjamin, Sara V Burns, Eric Novak, Amit Amin
OBJECTIVE: Length of stay (LOS) is a major driver of inpatient care costs. To date, few studies have investigated risk factors associated with increased LOS in patients with adult congenital heart disease (ACHD). In the present work, we sought to address this knowledge gap. METHODS: We conducted an analysis of the State Inpatient Databases from Arkansas, California, Florida, Hawaii, Nebraska and New York. We analysed data on admissions in patients with ACHD and constructed a series of hierarchical regression models to identify the clinical factors having the greatest effects on LOS...
February 25, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28237697/heart-transplantation-in-adults-with-congenital-heart-disease
#3
REVIEW
Lucile Houyel, Ngoc-Tram To-Dumortier, Yannick Lepers, Jérôme Petit, Régine Roussin, Mohamed Ly, Emmanuel Lebret, Elie Fadel, Jürgen Hörer, Sébastien Hascoët
With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients...
February 22, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28237536/early-experience-of-macitentan-for-pulmonary-arterial-hypertension-in-adult-congenital-heart-disease
#4
S Herbert, W Gin-Sing, L Howard, R M R Tulloh
BACKGROUND: Endothelin receptor antagonists (ERA) have been recognised as effective therapy for pulmonary arterial hypertension in congenital heart disease (CHD-PH), and Eisenmenger syndrome (ES) since the Breathe 5 study. A new dual receptor antagonist - Macitentan - is currently undergoing trials to determine its efficacy in simple ES. To date there is little information on this therapy in CHD and we report our first experience, some with more complex diseases. METHODS: Data was collected prospectively from September 2014...
February 6, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28236163/comparison-of-patients-undergoing-surgical-versus-transcatheter-pulmonary-valve-replacement-criteria-for-referral-and-mid-term-outcome
#5
Jenny E Zablah, Nilanjana Misra, Dorota Gruber, Dipak Kholwadwala, Shilpi Epstein
Pulmonary regurgitation and/or stenosis (PS) is challenging in patients with congenital heart defects. Our aim was to identify if criteria for referral were different between surgical (SPVR) and transcatheter pulmonary valve replacement (TPVR) populations, and to further assess if any baseline differences influence the resultant ventricular remodeling at medium-term follow-up. Retrospective chart review of patients post-SPVR or TPVR at our center from 2013 to 2015 was conducted. Volumetric data from cardiac magnetic resonance (CMR), 1 year before and 1 year after PVR was obtained...
February 25, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28229981/postprocedural-outcomes-and-risk-factors-for-arrhythmias-following-transcatheter-closure-of-congenital-perimembranous-ventricular-septal-defect-a-single-center-retrospective-study
#6
Li-Jian Zhao, Bo Han, Jian-Jun Zhang, Ying-Chun Yi, Dian-Dong Jiang, Jian-Li Lyu
BACKGROUND: Currently, transcatheter closure of perimembranous ventricular septal defect (pmVSD) is a widely accepted therapeutic modality. However, arrhythmias, especially postprocedural heart blocks, are a concern and outcomes are not very clear. This study explored the outcomes and risk factors of arrhythmias associated with transcatheter device closure of pmVSD. METHODS: A total of 395 patients diagnosed with pmVSD who successfully underwent transcatheter intervention between January 2010 and December 2015 in our center were retrospectively reviewed...
2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28228731/increased-hemodynamic-load-in-early-embryonic-stages-alters-endocardial-to-mesenchymal-transition
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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/28221263/use-of-model-based-iterative-reconstruction-to-improve-detection-of-congenital-cardiovascular-anomalies-in-infants-undergoing-free-breathing-computed-tomographic-angiography
#13
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
#14
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
#15
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/28211989/timothy-syndrome-1-genotype-without-syndactyly-and-major-extracardiac-manifestations
#16
Róbert Sepp, Lidia Hategan, Attila Bácsi, Judit Cseklye, László Környei, János Borbás, Márta Széll, Tamás Forster, István Nagy, Zoltán Hegedűs
Timothy syndrome 1 (TS1) is a rare genetic disorder characterized by multisystem abnormalities including QT prolongation, congenital heart defects, facial dysmorphism, episodic hypoglycemia, and neurological symptoms. A morphological hallmark of TS1 is syndactyly, present in all cases. TS1 is caused by the canonical p.Gly406Arg mutation in the alternatively spliced exon 8A in the CACNA1C gene, encoding for the main cardiac L-type calcium channel. A variant case of TS1 is reported. The proband had intermittent fetal bradycardia with heart rate of 72 bpm...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28211980/constitutional-bone-impairment-in-noonan-syndrome
#17
Giuseppina Baldassarre, Alessandro Mussa, Diana Carli, Cristina Molinatto, Giovanni Battista Ferrero
Noonan syndrome (NS) is an autosomal dominant trait characterized by genotypic and phenotypic variability. It belongs to the Ras/MAPK pathway disorders collectively named Rasopathies or neurocardiofaciocutaneous syndromes. Phenotype is characterized by short stature, congenital heart defects, facial dysmorphisms, skeletal and ectodermal anomalies, cryptorchidism, mild to moderate developmental delay/learning disability, and tumor predisposition. Short stature and skeletal dysmorphisms are almost constant and several studies hypothesized a role for the RAS pathway in regulating bone metabolism...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28211263/increased-regurgitant-flow-causes-endocardial-cushion-defects-in-an-avian-embryonic-model-of-congenital-heart-disease
#18
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/28208971/a-rare-association-of-pentalogy-of-fallot-with-situs-inversus-totalis-complicated-by-brain-abscess-in-an-adolescent-case-report
#19
Muhammed Basheer, Sunil Kumar Agarwalla
Tetralogy of Fallot (TOF) is the most frequently diagnosed congenital cyanotic heart disease. It is often associated with additional findings, such as atrial septal defect (i.e., pentalogy of Fallot) or right sided aortic arch. Association of this pentalogy of Fallot with situs inversus totalis is rarely reported in paediatric literature and it can cause technical challenges to intracardiac repair. We report the case of pentalogy of Fallot with dextrocardia and situs inversus presenting as parieto-occipital abscess in a 12-year-old child...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28208970/isolated-hypoplasia-of-left-pulmonary-artery-with-agenesis-of-left-lobe-of-thyroid-a-case-report
#20
Mohammed Abdul Khadir, Ganesh Narayana, Ganavi Ramagopal, Pradeep G Nayar
Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
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