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Congenital heart diseases

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https://www.readbyqxmd.com/read/27925466/a-prospective-evaluation-of-contrast-and-radiation-dose-and-image-quality-in-cardiac-ct-in-children-with-complex-congenital-heart-disease-using-low-concentration-iodinated-contrast-agent-and-low-tube-voltage-and-current
#1
Qiao-Ru Hou, Wei Gao, Yu Min Zhong, Ai Min Sun, Qian Wang, Hai Sheng Qiu, Fang Wang, Jian Ying Li, Liwei Hu
OBJECTIVE: To assess image quality, contrast dose and radiation dose in cardiac CT in children with congenital heart disease using low-concentration iodinated contrast agent and low tube voltage and current in comparison with standard-dose protocol. METHODS: 110 patients with congenital heart disease were randomized to one of the two scan protocols: Group A (n=45) with 120mA tube current and contrast agent of 270mgI/ml in concentration (Visipaque, GE Healthcare), and Group B (n=65) with the conventional 160mA and 370mgI/mL concentration contrast (Iopamiro, Bracco)...
December 7, 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27924361/prevalence-of-non-cardiovascular-findings-on-ct-angiography-in-children-with-congenital-heart-disease
#2
Archana Malik, Jeffrey C Hellinger, Sabah Servaes, Mathew C Schwartz, Marc S Keller, Monica Epelman
BACKGROUND: CT angiography is gaining broader acceptance in the evaluation of children with known or suspected congenital heart disease. These studies include non-cardiovascular structures such as the mediastinum, lung parenchyma and upper abdominal organs. It is important to inspect all these structures for potential abnormalities that might be clinically important and, in some cases, may impact care plans. OBJECTIVE: To determine the prevalence of non-cardiovascular findings in CT angiography of children with congenital heart disease...
December 6, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27919592/biomarkers-of-myocardial-injury-in-congenital-heart-disease-more-questions-than-answers
#3
EDITORIAL
Chun-An Chen
No abstract text is available yet for this article.
November 3, 2016: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/27914635/noninvasive-screening-for-pulmonary-hypertension-by-exercise-testing-in-congenital-heart-disease
#4
Jan Müller, Pinar Bambul Heck, Peter Ewert, Alfred Hager
BACKGROUND: Patients with congenital heart disease and native or palliated conditions are at risk to develop pulmonary hypertension (PH) in later life. Screening for PH is currently performed by regular echocardiographic follow-up, which appears to be difficult in several congenital conditions. This study evaluated the screening for PH in congenital heart disease by cardiopulmonary exercise testing (CPET). METHODS: We analyzed our database including all patients with congenital heart disease referred for CPET in our institution from June 2001 to September 2013 and identified 683 patients who had an accompanied heart catheterization less than 6 month after CPET...
November 30, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27913766/myocardial-cytochrome-oxidase-activity-increases-with-age-and-hypoxemia-in-patients-with-congenital-heart-disease
#5
Michael Onwugbufor, Richard J Levy, David Zurakowski, Richard A Jonas, Pranava Sinha
BACKGROUND: Myocardial tolerance to ischemia is influenced by age and preoperative cyanosis through unknown mechanisms and significantly affects postoperative outcomes. Cytochrome c oxidase (CcOx), the terminal enzyme of the mitochondrial electron transport chain, may play a role in the susceptibility to ischemic-reperfusion (IR) injury. Our study aimed at investigating changes in human myocardial CcOx activity based on age and preoperative oxygen saturation to understand its role in transition from neonatal to mature myocardium and hypoxic conditions...
December 1, 2016: Perfusion
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#6
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27912926/acute-management-of-refractory-and-unstable-pediatric-supraventricular-tachycardia
#7
Jonathan Lewis, Gaurav Arora, Dana L Tudorascu, Robert W Hickey, Richard A Saladino, Mioara D Manole
OBJECTIVE: To characterize the management of acute pediatric supraventricular tachycardia (SVT), placing special emphasis on infants, patients refractory to adenosine (refractory SVT), and patients with hypotension, poor perfusion, or altered mental status (unstable SVT). STUDY DESIGN: Retrospective cohort study of patients 0-18 years of age without congenital heart disease who presented to our pediatric hospital from January 2003 to December 2012 for the treatment of acute SVT...
November 22, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27912205/the-prevalence-of-sexual-dysfunction-and-its-association-with-quality-of-life-in-adults-with-congenital-heart-disease
#8
Alexandra Neiman, Salil Ginde, Michael G Earing, Peter J Bartz, Scott Cohen
BACKGROUND: The prevalence of sexual dysfunction (SD) and its impact on quality of life (QOL) in adults with congenital heart disease (CHD) is not well known. The aims of this study were to: determine the prevalence of SD, evaluate the risk factors associated with SD, and determine the association between SD and QOL in adults with CHD. METHODS: This was a cross-sectional study of adults (≥18years) with CHD presenting for routine follow-up at our institution. Subjects completed the CDC HRQOL-14 "Health Days Measure" to assess mental and physical health, and either the Sexual Health Inventory for Men or the Female Sexual Function Index to assess sexual function...
November 19, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27911332/type-0-spinal-muscular-atrophy-further%C3%A2-delineation-of-prenatal-and%C3%A2-postnatal-features-in-16-patients
#9
Sarah Grotto, Jean-Marie Cuisset, Stéphane Marret, Séverine Drunat, Patricia Faure, Séverine Audebert-Bellanger, Isabelle Desguerre, Vincent Flurin, Anne-Gaëlle Grebille, Anne-Marie Guerrot, Hubert Journel, Gilles Morin, Ghislaine Plessis, Sylvain Renolleau, Joëlle Roume, Brigitte Simon-Bouy, Renaud Touraine, Marjolaine Willems, Thierry Frébourg, Eric Verspyck, Pascale Saugier-Veber
BACKGROUND: Spinal muscular atrophy (SMA) is caused by homozygous inactivation of the SMN1 gene. The SMN2 copy number modulates the severity of SMA. The 0SMN1/1SMN2 genotype, the most severe genotype compatible with life, is expected to be associated with the most severe form of the disease, called type 0 SMA, defined by prenatal onset. OBJECTIVE: The aim of the study was to review clinical features and prenatal manifestations in this rare SMA subtype. METHODS: SMA patients with the 0SMN1/1SMN2 genotype were retrospectively collected using the UMD-SMN1 France database...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27909479/atrial-fibrillation-ablation-in-adults-with-repaired-congenital-heart-disease
#10
REVIEW
Marta Acena Md, Ignasi Anguera Md PhD, Paolo D Dallaglio Md, Marcos Rodriguez Md, Xavier Sabaté Md PhD
The incidence of atrial fibrillation (AF) in congenital heart disease (CHD) adults has increased in the past decades due to a longer life expectancy of this population where the subjects are exposed to cardiac overflow, overpressure and structural changes for years. The literature regarding AF ablation in repaired CHD adults emphasizes the importance of intracardiac echocardiography (ICE) to perform the transseptal puncture and the ablation procedure in the left atrium (LA), both effectively and safely. In small case control studies, where the predominant congenital cardiomyopathy was the atrial septal defect, the most common strategy for ablation was antral isolation of the pulmonary veins showing results, at one year follow-up, similar to those in the general population...
February 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27908445/erratum-for-lesion-specific-factors-contributing-to-in-hospital-costs-in-adults-with-congenital-heart-disease-am-j-cardiol-2016-117-1821-1825
#11
(no author information available yet)
No abstract text is available yet for this article.
November 28, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/27907889/denovo-db-a-compendium-of-human-de-novo-variants
#12
Tychele N Turner, Qian Yi, Niklas Krumm, John Huddleston, Kendra Hoekzema, Holly A F Stessman, Anna-Lisa Doebley, Raphael A Bernier, Deborah A Nickerson, Evan E Eichler
Whole-exome and whole-genome sequencing have facilitated the large-scale discovery of de novo variants in human disease. To date, most de novo discovery through next-generation sequencing focused on congenital heart disease and neurodevelopmental disorders (NDDs). Currently, de novo variants are one of the most significant risk factors for NDDs with a substantial overlap of genes involved in more than one NDD. To facilitate better usage of published data, provide standardization of annotation, and improve accessibility, we created denovo-db (http://denovo-db...
October 5, 2016: Nucleic Acids Research
https://www.readbyqxmd.com/read/27907850/automated-annotation-and-quantitative-description-of-ultrasound-videos-of-the-fetal-heart
#13
Christopher P Bridge, Christos Ioannou, J Alison Noble
Interpretation of ultrasound videos of the fetal heart is crucial for the antenatal diagnosis of congenital heart disease (CHD). We believe that automated image analysis techniques could make an important contribution towards improving CHD detection rates. However, to our knowledge, no previous work has been done in this area. With this goal in mind, this paper presents a framework for tracking the key variables that describe the content of each frame of freehand 2D ultrasound scanning videos of the healthy fetal heart...
November 19, 2016: Medical Image Analysis
https://www.readbyqxmd.com/read/27906824/selection-of-patients-for-initial-clinical-trials-of-solid-organ-xenotransplantation
#14
David K C Cooper, Martin Wijkstrom, Sundaram Hariharan, Joshua L Chan, Avneesh Singh, Keith Horvath, Muhammad Mohiuddin, Arielle Cimeno, Rolf N Barth, John C LaMattina, Richard N Pierson
Several groups have reported extended survival of genetically-engineered pig organs in nonhuman primates, varying from almost 10 months for life-supporting kidney grafts and >2 years for nonlife-supporting heart grafts to less than 1 month for life-supporting liver and lung grafts. We have attempted to define groups of patients who may not have an option to wait for an allograft. These include kidney, heart, and lung candidates who are highly-allosensitized. In addition, some kidney candidates (who have previously lost at least 2 allografts from rapid recurrence of native kidney disease) have a high risk of further recurrence and will not be offered a repeat allotransplant...
December 1, 2016: Transplantation
https://www.readbyqxmd.com/read/27906085/tissue-engineered-cardiac-patch-seeded-with-human-induced-pluripotent-stem-cell-derived-cardiomyocytes-promoted-the-regeneration-of-host-cardiomyocytes-in-a-rat-model
#15
Tadahisa Sugiura, Narutoshi Hibino, Christopher K Breuer, Toshiharu Shinoka
BACKGROUND: Thousands of babies are born with congenital heart defects that require surgical repair involving a prosthetic implant. Lack of growth in prosthetic grafts is especially detrimental in pediatric surgery. Cell seeded biodegradable tissue engineered grafts are a novel solution to this problem. The purpose of the present study is to evaluate the feasibility of seeding human induced pluripotent stem cell derived cardiomyocytes (hiPS-CMs) onto a biodegradable cardiac patch. METHODS: The hiPS-CMs were cultured on a biodegradable patch composed of a polyglycolic acid (PGA) and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer (PLCL) for 1 week...
December 1, 2016: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27905645/-morbidity-profile-among-adolescents-with-down-syndrome
#16
Antonia Cárdenas, María A Paul, Catalina Correa, Sebastián Valderrama, Jaime Cerda, Macarena Lizama
BACKGROUND: There is a paucity of information about morbidity and mortality of adolescents with Down syndrome (DS). AIM: To describe morbidity and mortality of a cohort of Chilean adolescents with DS. MATERIAL AND METHODS: Review of electronic clinical records of 67 ambulatory patients with DS aged 10 to 20 years (37 women), seen between the years 2007 and 2014 in outpatient clinics of a University hospital. RESULTS: The mean age at the last consultation was 13 ± 3 years...
August 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/27904846/complete-congenital-heart-block-in-a-neonate-with-a-complex-congenital-heart-defect-in-africa
#17
Clovis Nkoke, Edvine Yonta Wawo, Liliane Kuate Mfeukeu, Larissa Makamte, Sandrine Dikosso Edie, Flore Esiene Balana
Congenital heart block (CHB) is rare disorder that has a higher mortality when associated with structural congenital heart defects. Very few cases have been reported in Sub-Saharan Africa (SSA). We present a case of complete CHB associated with a complex congenital heart defect in a neonate in Cameroon. A 1-month-old neonate in Cameroon was referred for the evaluation of bradycardia. The obstetrical ultrasound done during pregnancy revealed fetal bradycardia without further evaluation. Clinical examination showed well a developed neonate with bradycardia at 62 beats/minute, and mild cyanosis with oxygen saturation at 93% at room air...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27904843/cardiac-surgery-in-africa-a-thirty-five-year-experience-on-open-heart-surgery-in-cote-d-ivoire
#18
Koffi Herve Yangni-Angate, Christophe Meneas, Florent Diby, Manga Diomande, Anicet Adoubi, Yves Tanauh
BACKGROUND: Few centers for open heart surgery (OHS) are in Sub-Saharan Africa. Lack of OHS results is also noted. By reporting our African experience on OHS, the aim of this study was to fill the gap. METHODS: It is a retrospective study on 2,612 patients who were subject to an OHS between 1978 and 2013. Data were collected from demographical, clinical, investigative studies, surgical and outcomes parameters. RESULTS: There were 1,475 cases of rheumatic heart diseases (RHD), 126 endomyocardial fibrosis (EMF), 741 congenital heart diseases (CHDs) and 270 various affections...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27904842/the-challenges-of-cardiothoracic-surgery-practice-in-nigeria-a-12-years-institutional-experience
#19
Bode Falase, Michael Sanusi, Adeola Animasahun, Ogadinma Mgbajah, Adetinuwe Majekodunmi, Onyekwelu Nzewi, Jonathan Nwiloh, David Oke
BACKGROUND: Although the specialty of cardiothoracic surgery has been practiced in Nigeria for many years, open heart surgery (OHS) has only in the last decade become relatively more frequent, mainly through visiting foreign cardiac surgical teams. At this early phase of development it is faced with multiple challenges, especially financing and local skilled manpower for which solutions have to be identified in order to ensure sustainability and future growth. This study is aimed at highlighting these obstacles to growth of cardiothoracic surgery based on our own institutional experience at Lagos State University Teaching Hospital (LASUTH) and the current status of OHS activity in other cardiothoracic centers in Nigeria...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27904841/the-rebirth-of-cardiac-surgery-in-sudan
#20
Ahmed ElSayed, Elamin Elawad Elnur
BACKGROUND: Cardiac surgery is nowadays an integral part of a complete national health service. METHODS: In this paper the rebirth of cardiac surgery in Sudan from 1998 till 2007 is given by means of a literature search, personal interviews and searching hospital records. RESULTS: A summary of the initial birth of these services in 1959 till its demise in 1993 is first mentioned to set the stage for the subsequent period of sustainable growth...
October 2016: Cardiovascular Diagnosis and Therapy
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