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Congenital cardiac

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https://www.readbyqxmd.com/read/28214968/piloting-the-use-of-patient-specific-cardiac-models-as-a-novel-tool-to-facilitate-communication-during-cinical-consultations
#1
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
#2
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
https://www.readbyqxmd.com/read/28212480/-reintervention-with-percutaneous-balloon-angioplasty-in-patients-with-congenital-heart-disease-with-left-sided-obstructions
#3
Horacio Márquez-González, Diana López-Gallegos, Nataly Alejandra Pérez-Velázquez, Lucelli Yáñez-Gutiérrez
BACKGROUND: Left-sided cardiac obstructions represent 15% of congenital heart disease (CHD). The treatment in adults is surgical; however, balloon dilation by interventional catheterization can alleviate the symptoms in pediatric patients to allow them to reach the target height. The aim was to determine the survival and the factors associated with reintervention in patients with CHD with left-sided obstruction treated with balloon angioplasty. METHODS: A cohort study was conducted in patients aged 4 to 17 years with left-sided heart obstruction (valvular stenosis [VS], supravalvular aortic stenosis [SAS], coarctation of the aorta [CA]) successfully treated with balloon angioplasty...
2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28212053/transthoracic-echocardiography-pitfalls-and-limitations-as-delineated-at-cardiac-ct-and-mr-imaging
#4
Sachin B Malik, Natalie Chen, Rex A Parker, Joe Y Hsu
Transthoracic echocardiography ( TTE transthoracic echocardiography ) is a critical tool in the field of clinical cardiology. It often serves as one of the first-line imaging modalities in the evaluation of cardiac disease owing to its low cost, portability, widespread availability, lack of ionizing radiation, and ability to evaluate both anatomy and function of the heart. Consequently, a large majority of patients undergoing a cardiac computed tomography (CT) or magnetic resonance (MR) imaging examination will have a TTE transthoracic echocardiography available for review...
February 17, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28211989/timothy-syndrome-1-genotype-without-syndactyly-and-major-extracardiac-manifestations
#5
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/28211263/increased-regurgitant-flow-causes-endocardial-cushion-defects-in-an-avian-embryonic-model-of-congenital-heart-disease
#6
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
#7
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/28208963/clinical-profile-of-cardiac-arrhythmias-in-children-attending-the-out-patient-department-of-a-tertiary-paediatric-care-centre-in-chennai
#8
Sarala Premkumar, Premkumar Sundararajan, Thangavelu Sangaralingam
INTRODUCTION: The presentation of symptoms of paediatric arrhythmias vary depending on the age and underlying heart disease. Physical examination of children with important arrhythmias may be entirely normal. AIM: Aim is to study the characteristics of cardiac arrhythmias in paediatric patients in a tertiary paediatric care centre in Chennai, India. MATERIALS AND METHODS: The participants (n=60) were from birth to 12 years of age. Patients with sinus arrhythmias, sinus tachycardia and sinus bradycardia were excluded...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28205344/utility-of-a-standardized-postcardiopulmonary-bypass-epicardial-echocardiography-protocol-for-stage-i-norwood-palliation
#9
Kenan W D Stern, Kimberlee Gauvreau, Sitaram Emani, Tal Geva
OBJECTIVE: Stage 1 Norwood palliation is one of the highest risk procedures in congenital cardiac surgery. Patients with superior technical performance scores have more favorable outcomes. Intraoperative epicardial echocardiography may allow the surgeon to address residual lesions prior to leaving the operating room, resulting in improved technical performance. The ability of intraoperative epicardial echocardiography to visualize the relevant anatomy and its association with outcomes is not known...
February 16, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28203572/symptomatic-type-iv-dual-left-anterior-descending-coronary-artery
#10
Kyriacos Papadopoulos, Georgios M Georgiou, Evagoras Nicolaides
Dual left anterior descending coronary artery is a rare congenital anomaly with 4 subtypes. Double left anterior descending coronary artery originating from the left main stem and the right coronary artery (type IV dual left anterior descending artery) has been reported to occur in 0.01% to 0.7% of patients undergoing cardiac catheterization. We report a case of a 49-year-old woman who was found to have this anomaly during coronary angiography. The patient had been complaining of chest pain that mimics angina pectoris and exercise tolerance test was positive for myocardial ischemia...
October 2016: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28202702/congenital-myopathy-associated-with-the-triadin-knockout-syndrome
#11
Andrew G Engel, Keeley R Redhage, David J Tester, Michael J Ackerman, Duygu Selcen
OBJECTIVE: Triadin is a component of the calcium release complex of cardiac and skeletal muscle. Our objective was to analyze the skeletal muscle phenotype of the triadin knockout syndrome. METHODS: We performed clinical evaluation, analyzed morphologic features by light and electron microscopy, and immunolocalized triadin in skeletal muscle. RESULTS: A 6-year-old boy with lifelong muscle weakness had a triadin knockout syndrome caused by compound heterozygous null mutations in triadin...
February 15, 2017: Neurology
https://www.readbyqxmd.com/read/28198573/radiation-dose-benchmarks-in-pediatric-cardiac-catheterization-a-prospective-multi-center-c3po-qi-study
#12
Priscila C Cevallos, Aimee K Armstrong, Andrew C Glatz, Bryan H Goldstein, Todd M Gudausky, Ryan A Leahy, Christopher J Petit, Shabana Shahanavaz, Sara M Trucco, Lisa J Bergersen
OBJECTIVES: This study sought to update benchmark values to use a quality measure prospectively. BACKGROUND: Congenital Cardiac Catheterization Outcomes Project - Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014. METHODS: Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015...
February 15, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28197939/prenatal-counseling-of-fetal-congenital-heart-disease
#13
REVIEW
Caroline K Lee
The field of fetal cardiology has advanced greatly over the last two decades and congenital heart defects can now be identified in utero with a high level of accuracy. Prenatal counseling of parents given the news of a fetal cardiac defect is an important role of the fetal cardiologist. Prenatal counseling is a complex task that requires skill to perform and interpret fetal echocardiograms, an understanding of fetal and postnatal cardiovascular physiology, knowledge of therapeutic and surgical options, and of long-term outcomes including quality of life...
January 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28197808/age-related-alterations-of-the-cd19-complex-and-memory-b-cells-in-children-with-down-syndrome
#14
Ayse Nazli Seckin, Hulya Ozdemir, Ayca Ceylan, Hasibe Artac
Children with Down syndrome (DS) have a high incidence of recurrent respiratory tract infections, leukaemia and autoimmune disorders, suggesting immune dysfunction. The present study evaluated the role of the CD19 complex and memory B cells in the pathogenesis of immunodeficiency in children with DS. The expression levels (median fluorescein intensity-MFI) of CD19, CD21 and CD81 molecules on the surface of B cells and memory B cell subsets were studied in 37 patients and 39 healthy controls. Twenty-nine of the DS group had congenital cardiac disease...
February 14, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/28197291/postoperative-right-ventricular-failure-in-cardiac-surgery
#15
REVIEW
Victor H Nieto Estrada, Daniel L Molano Franco, Albert A Valencia Moreno, Jose A Rojas Gambasica, Cristian C Cortes Nunez
Two cases of patients that developed right ventricular failure (RVF) after cardiac valve surgery are presented with a narrative revision of the literature. RVF involves a great challenge due to the severity of this condition; it has a low incidence among non-congenital cardiac surgery patients, is more likely associated with cardiovascular and pulmonary complications related to cardiopulmonary bypass (CPB), and is a cause of acute graft failure and of a higher early mortality in cardiac transplant. The morphologic and hemodynamic characteristics of the right ventricle and some specific factors that breed pulmonary hypertension after cardiac surgery are in favor of the onset of RVF...
December 2016: Cardiology Research
https://www.readbyqxmd.com/read/28197280/predictors-of-permanent-pacemaker-implantation-after-coronary-artery-bypass-grafting-and-valve-surgery-in-adult-patients-in-current-surgical-era
#16
Bandar Al-Ghamdi, Yaseen Mallawi, Azam Shafquat, Alexandra Ledesma, Nadiah AlRuwaili, Mohamed Shoukri, Shahid Khan, Aly Al Sanei
BACKGROUND: Permanent pacemaker (PPM) implantation after cardiac surgery is required in 0.4-6% of patients depending on cardiac surgery type. PPM implantation in the early postoperative period may reduce morbidity and postoperative hospital stay. We performed a retrospective review of electronic medical records of adult patients with coronary artery bypass grafting (CABG), valve surgery, or both, over a 3-year period. Our aim was to identify predictors of PPM requirements and PPM dependency on follow-up in the current surgical era...
August 2016: Cardiology Research
https://www.readbyqxmd.com/read/28196901/novel-variant-in-the-ank2-membrane-binding-domain-is-associated-with-ankyrin-b-syndrome-and-structural-heart-disease-in-a-first-nations-population-with-a-high-rate-of-long-qt-syndrome
#17
Leigh Anne Swayne, Nathaniel P Murphy, Sirisha Asuri, Lena Chen, Xiaoxue Xu, Sarah McIntosh, Chao Wang, Peter J Lancione, Jason D Roberts, Charles Kerr, Shubhayan Sanatani, Elizabeth Sherwin, Crystal F Kline, Mingjie Zhang, Peter J Mohler, Laura T Arbour
BACKGROUND: Long QT syndrome confers susceptibility to ventricular arrhythmia, predisposing to syncope, seizures, and sudden death. While rare globally, long QT syndrome is ≈15× more common in First Nations of Northern British Columbia largely because of a known mutation in KCNQ1. However, 2 large multigenerational families were affected, but negative for the known mutation. METHODS AND RESULTS: Long QT syndrome panel testing was carried out in the index case of each family, and clinical information was collected...
January 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28196823/cor-triatriatum-sinistrum-presentation-of-syncope-and-atrial-tachycardia
#18
Malcolm Avari, Sunil Nair, Zofia Kozlowska, Samer Nashef
We present a rather unusual cause for syncope associated with atrial tachycardia. A man aged 39 years presented with an episode of syncope and narrow complex tachycardia. Further investigations, including transoesophageal echocardiography, identified cor triatriatum sinistrum (CTS), a rare congenital abnormality characterised by the atrium being divided by a fibrous membrane. Although it is rare, there has been an increase in diagnosis due to developments in diagnostic imaging techniques. Symptoms are related to the size of fenestrations within the fibrous membrane...
February 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28196557/anomalous-drainage-of-isolated-superior-caval-vein-to-the-left-atrium-the-oldest-reported-unrepaired-case
#19
Maria Boutsikou, Dudley J Pennell, Eva Nyktari
Isolated right superior caval vein drainage into the left atrium is a rare congenital cardiac anomaly usually presenting with hypoxaemia and cyanosis. Surgical repair is the definitive treatment for this condition. In this report, we present the case of a 72-year-old patient with uncorrected anomalous drainage of a right superior caval vein into the left atrium, first diagnosed in the 1960s. To the best of our knowledge, this is the oldest reported unrepaired case.
February 15, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28194611/using-three-dimensional-echocardiography-to-guide-left-ventricle-lead-position-in-cardiac-resynchronization-therapy-does-it-make-any-difference
#20
Haitham A Badran, John Z Kamel, Tarek R Mohamed, Mohamed A Abdelhamid
BACKGROUND: Cardiac resynchronization therapy (CRT) is an effective treatment for patients with advanced heart failure. Nearly 30% of candidates are inadequate responders. Proper patient selection, left ventricle (LV) lead placement optimization, and optimization of the programming of the CRT device are important approaches to improve outcome of CRT. We examined the role of three-dimensional (3D) echocardiography in determining the optimal LV lead position as a method of optimizing CRT response...
February 13, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
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