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Congenital cardiac defect

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https://www.readbyqxmd.com/read/28730319/management-of-postoperative-junctional-ectopic-tachycardia-in-pediatric-patients-a-survey-of-30-centers-in-germany-austria-and-switzerland
#1
Andreas Entenmann, Miriam Michel, Ulrike Herberg, Nikolaus Haas, Matthias Kumpf, Matthias Gass, Friedemann Egender, Roman Gebauer
Postoperative junctional ectopic tachycardia (JET) is a frequent complication after pediatric cardiac surgery. Current recommendations on how and when to treat JET are inconsistent. We evaluated the management strategies of postoperative JET in German-speaking countries. We sent an online survey to 30 centers of pediatric cardiology that perform surgery for congenital heart defects in Germany (24), Austria (4), and Switzerland (2). The survey asked 18 questions about how and in what treatment sequence postoperative JET was managed...
July 21, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28730300/health-related-quality-of-life-in-paediatric-patients-with-congenital-heart-defects-association-with-the-type-of-heart-defect-and-the-surgical-technique
#2
A Heusch, H J Kahl, K O Hensel, G Calaminus
The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL. METHODS: Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected...
July 20, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28726068/cardiac-complications-of-congenital-disorders-of-glycosylation-cdg-a-systematic-review-of-the-literature
#3
REVIEW
D Marques-da-Silva, R Francisco, D Webster, V Dos Reis Ferreira, J Jaeken, T Pulinilkunnil
Congenital disorders of glycosylation (CDG) are inborn errors of metabolism due to protein and lipid hypoglycosylation. This rapidly growing family of genetic diseases comprises 103 CDG types, with a broad phenotypic diversity ranging from mild to severe poly-organ -system dysfunction. This literature review summarizes cardiac involvement, reported in 20% of CDG. CDG with cardiac involvement were divided according to the associated type of glycosylation: N-glycosylation, O-glycosylation, dolichol synthesis, glycosylphosphatidylinositol (GPI)-anchor biosynthesis, COG complex, V-ATPase complex, and other glycosylation pathways...
July 19, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28723882/altered-carnitine-homeostasis-in-children-with-increased-pulmonary-blood-flow-due-to-ventricular-septal-defects
#4
Stephen M Black, Aida Field-Ridley, Shruti Sharma, Sanjiv Kumar, Roberta L Keller, Rebecca Kameny, Emin Maltepe, Sanjeev A Datar, Jeffrey R Fineman
OBJECTIVES: Congenital heart disease with increased pulmonary blood flow results in progressive pulmonary vascular endothelial dysfunction and associated increased perioperative morbidity. Using our ovine model of congenital heart disease with increased pulmonary blood flow, we have previously demonstrated progressive endothelial dysfunction associated with disruption in carnitine homeostasis, mitochondrial dysfunction, decreased nitric oxide signaling, and enhanced reactive oxygen species generation...
July 18, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28722171/use-of-antibiotics-during-pregnancy-and-the-risk-of-major-congenital-malformations-a-population-based-cohort-study
#5
Flory T Muanda, Odile Sheehy, Anick Bérard
INTRODUCTION: Few studies have investigated the link between individual antibiotics and major congenital malformations (MCMs) including specific malformations owing to small sample size. We aimed to quantify the association between exposure to gestational antibiotic and the risk of MCMs. METHODS: Using the Quebec pregnancy cohort (1998 -2008), we included a total of 139,938 liveborn singleton alive whose mothers were covered by the «Régie de l'assurance maladie du Québec" drug plan for at least 12 months before and during pregnancy...
July 19, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28720136/bilateral-multiple-pulmonary-artery-aneurysms-associated-with-cavitary-pulmonary-tuberculosis-a-case-report
#6
Pedro Pallangyo, Frederick Lyimo, Smita Bhalia, Hilda Makungu, Bashir Nyangasa, Flora Lwakatare, Pal Suranyi, Mohamed Janabi
BACKGROUND: Pulmonary artery aneurysms constitute <1% of aneurysms occurring in the thoracic cavity. Congenital cardiac defects are responsible for the majority (>50%) of cases, however, pulmonary artery aneurysm is a rare sequelae of pulmonary tuberculosis reported in about 5% of patients with chronic cavitary tuberculosis on autopsy. The natural history of this potentially fatal condition remains poorly understood and guidelines for optimal management are controversial. CASE PRESENTATION: A 24-year-old man, a nursing student of African descent, was referred to us from an up-country regional hospital with a 4-week history of recurrent episodes of breathlessness, awareness of heartbeats and coughing blood 3 weeks after completing a 6-month course of anti-tuberculosis drugs...
July 19, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#7
Constanze Pfitzer, Paul C Helm, Hannah Ferentzi, Lisa-Maria Rosenthal, Ulrike M M Bauer, Felix Berger, Katharina R L Schmitt
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions)...
July 18, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28713984/downregulation-of-microrna%C3%A2-199a%C3%A2-5p-protects-cardiomyocytes-in-cyanotic-congenital-heart-disease-by-attenuating-endoplasmic-reticulum-stress
#8
Yang Zhou, Wei-Kun Jia, Zhao Jian, Liang Zhao, Chen-Cheng Liu, Yong Wang, Ying-Bin Xiao
Chronic hypoxia is a key pathological change in patients with cyanotic congenital heart defect (CCHD). It has been demonstrated that enhanced myocardial unfolded protein response (UPR) increases the capacity to buffer endoplasmic reticulum (ER) stress and to avoid subsequent apoptosis caused by the hypoxia that underlies CCHD. The present study was performed to determine the regulatory role of microRNAs (miRNAs) in this cytoprotective UPR process. The results revealed that miR‑199a‑5p was markedly downregulated in the cardiac tissue of patients with CCHD and in human myocardial cells cultured in hypoxic conditions...
July 6, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28709918/unroofed-coronary-sinus-syndrome-an-easily-corrected-congenital-anomaly-but-more-diagnostic-suspicions-are-needed
#9
Changcheng Chen, Lili Xu, Yi Xu, Ping Li, Shuo Liu, Bin You
BACKGROUND: Unroofed coronary sinus syndrome (URCSS) is a spectrum of cardiac anomalies in which part (partial type) or the entire common wall (complete type) between the coronary sinus (CS) and left atrium is absent. It is commonly associated with a persistent left superior vena cava (PLSVC). The PLSVC can even anomalously connect to the left atrium in complete type anomaly. URCSS has been reported to be associated with delayed diagnosis and life-threatening cerebral injury. The purpose of the present study was to review our experience with surgical correction of this often-concealed malformation and discuss methods for reducing diagnostic omission...
June 22, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28707420/transthoracic-intracardiac-catheters-in-pediatric-cardiac-patients-a-single-center-experience
#10
Kristoffer Beham, Hitendu Dave, Janet Kelly, Bernhard Frey, Maja I Hug, Barbara Brotschi
BACKGROUND: Transthoracic intracardiac catheters are frequently inserted in children during congenital heart surgery for monitoring and vascular access purposes. Their use entails a small potential risk. AIM: We aimed to evaluate both catheter-associated morbidities related to maintenance and removal of transthoracic intracardiac catheters in pediatric cardiac patients, and predictors for catheter-associated adverse events. METHODS: Single-center retrospective cohort study of prospectively collected data of children aged 0-14 years receiving a transthoracic intracardiac catheter inserted in the operating room during 7 consecutive years at the University Children's Hospital Zurich...
July 14, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28706585/atrial-tachyarrhythmia-in-adult-congenital-heart-disease
#11
REVIEW
Arsha Karbassi, Krishnakumar Nair, Louise Harris, Rachel M Wald, S Lucy Roche
The adult congenital heart disease (ACHD) population continues to grow and most cardiologists, emergency room physicians and family doctors will intermittently come into contact with these patients. Oftentimes this may be in the setting of a presentation with atrial tachyarrhythmia; one of the commonest late complications of ACHD and problem with potentially serious implications. Providing appropriate initial care and ongoing management of atrial tachyarrhythmia in ACHD patients requires a degree of specialist knowledge and an awareness of certain key issues...
June 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28704916/three-dimensional-echocardiography-in-adult-congenital-heart-disease
#12
REVIEW
Hyun Suk Yang
Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions...
July 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28701611/the-utility-of-targeted-perioperative-transthoracic-echocardiography-in-managing-an-adult-patient-with-anomalous-origin-of-the-left-coronary-artery-pulmonary-artery-for-noncardiac-surgery
#13
Anudeep Jafra, Suman Arora, Aveek Jayant
Congenital coronary artery anomalies as a whole are uncommon. Abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is probably the most common congenital coronary defect. An overwhelming majority of the patients with untreated ALCAPA do not survive to adulthood. As yet, there is no consensus on the management of adults with ALCAPA. We describe a patient with breast malignancy and incidentally detected ALCAPA; primacy was given to treatment of the oncologic condition as a first step...
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28701211/inpatient-rehabilitation-for-adult-patients-with-marfan-syndrome-an-observational-pilot-study
#14
Dieter Benninghoven, Denise Hamann, Yskert von Kodolitsch, Meike Rybczynski, Julia Lechinger, Friedrich Schroeder, Marina Vogler, Eike Hoberg
BACKGROUND: Advances in medical, interventional and surgical treatment have increased average life expectancy of patients with congenital heart defects. As a result a new group of adult patients with congenital cardiac defects requires medical rehabilitation. Patients with Marfan syndrome (MFS) are a relevant group among these patients. So far, no reports on the effectiveness of specialized rehabilitation programmes for MFS patients exist. We implemented an inpatient 3-week rehabilitation program for MFS patients at the Muehlenberg-Clinic for rehabilitation and assessed the medical safety as well as the impact of the program on physical fitness and psychological wellbeing of participants by means of an observational pilot study...
July 12, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28697788/anatomic-histopathologic-and-echocardiographic-features-in-a-dog-with-an-atypical-pulmonary-valve-stenosis-with-a-fibrous-band-of-tissue-and-a-patent-ductus-arteriosus
#15
Hakyoung Yoon, Jaehwan Kim, Sang-Soep Nahm, Kidong Eom
BACKGROUND: Congenital pulmonary valve stenosis and patent ductus arteriosus are common congenital heart defects in dogs. However, concurrence of atypical pulmonary valve stenosis and patent ductus arteriosus is uncommon. This report describes the anatomic, histopathologic, and echocardiographic features in a dog with concomitant pulmonary valve stenosis and patent ductus arteriosus with atypical pulmonary valve dysplasia that included a fibrous band of tissue. CASE PRESENTATION: A 1...
July 11, 2017: Acta Veterinaria Scandinavica
https://www.readbyqxmd.com/read/28696220/prevention-of-sudden-cardiac-death-in-adults-with-congenital-heart-disease-do-the-guidelines-fall-short
#16
Jim T Vehmeijer, Zeliha Koyak, Werner Budts, Louise Harris, Candice K Silversides, Erwin N Oechslin, Berto J Bouma, Aeilko H Zwinderman, Barbara J M Mulder, Joris R de Groot
BACKGROUND: Sudden cardiac death (SCD) is a major cause of mortality in adult congenital heart disease (ACHD) patients. SCD may be prevented by implantable cardioverter-defibrillator (ICD) implantation, but patient stratification remains troublesome. The 2014 Consensus Statement on Arrhythmias in ACHD patients and the 2015 European Society of Cardiology Guidelines specified recommendations for ICD implantation in ACHD patients for the first time. We assess the discriminative ability of these ICD recommendations for SCD in ACHD patients...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28690994/delayed-diagnosis-of-22q11-deletion-syndrome-due-to-late-onset-hypocalcemia-in-a-11-year-old-girl-with-imperforated-anus
#17
Dong-Yoon Yoo, Hae Jung Kim, Kee Hyun Cho, Eun Byul Kwon, Eun-Gyong Yoo
Neonatal hypocalcemia and congenital heart defects has been known as the first clinical manifestation of the chromosome 22q11.2 deletion syndrome (22q11DS). However, because of its wide clinical spectrum, diagnosis of 22q11DS can be delayed in children without classic symptoms. We report the case of a girl with the history of imperforate anus but without neonatal hypocalcemia or major cardiac anomaly, who was diagnosed for 22q11DS at the age of 11 after the onset of overt hypocalcemia. She was born uneventfully from phenotypically normal Korean parents...
June 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28690953/venous-diversion-surgery-revisited-a-baffling-situation
#18
Jessica R Klassen, Davinder S Jassal, Brett Memauri, Malek Kass, James W Tam, Jonathan Windram, David Ross, Nasir Shaikh
With the increasing number of survivors with congenital heart disease (CHD) reaching adulthood, it is important for the clinician to be familiar with the various surgical options performed in this growing patient population. We describe the case of a 65-year-old female who presented with hypoxia and right-to-left shunting following a surgical repair of an atrial septal defect (ASD) secundum and anomalous pulmonary veins with a partial atrial diversion procedure in childhood. The use of multimodality cardiovascular imaging using echocardiography, computed tomography, magnetic resonance imaging, and invasive cardiac catheterization was complementary in the preoperative diagnosis and management of this unique baffling situation...
June 6, 2017: Curēus
https://www.readbyqxmd.com/read/28690218/-comparative-study-between-cardiac-catheterization-intervention-therapy-and-transthoracic-small-incision-surgery-for-closure-of-congenital-atrial-septal-defect-by-domestic-occluder-with-echocardiographic-monitoring
#19
Xiaomei He, Lina Zhao, Xuejia Guo, Ning Zhang, Yuna Sun, Jun Wang, Zhen Wang, Gaiqin Liu
To evaluate the safety of cardiac catheterization intervention therapy and transthoracic small incision surgery in the occlusion bydomestic occluder under echocardiography guiding in patients with atrial septal defect (ASD).
 Methods: A total of 1 080 patients with ASD in the occlusion by domestic occluder were analyzed retrospectively, and the interventional treatment were performed in 734 cases through cardiac catheterization intervention therapy and 346 cases through transthoracic small incision surgery...
June 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28681132/lower-hospital-charges-and-societal-costs-for-catheter-device-closure-of-atrial-septal-defects
#20
Jessica N Sanchez, Michael D Seckeler
Atrial septal defects (ASD) are among the most common congenital heart defects. As more ASDs are corrected by interventional catheterization instead of surgery, it is critical to understand the associated clinical and societal costs. The goal of this study was to use a national U.S. database to describe hospital charges and societal costs for surgical and catheter-based (ASD) closure. Retrospective review of hospital discharge data from the Kids' Inpatient Database from January 2010 to December 2012. The database was queried for admissions for <21 years old with ICD-9 procedure codes for surgical (35...
July 5, 2017: Pediatric Cardiology
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