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pediatric congenital heart diseases

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https://www.readbyqxmd.com/read/29152906/short-term-outcomes-following-implementation-of-a-dedicated-young-adult-congenital-heart-disease-transition-program
#1
Sumeet S Vaikunth, Roberta G Williams, Merujan Y Uzunyan, Han Tun, Cheryl Barton, Philip M Chang
OBJECTIVE: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease...
November 20, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29151308/unknown-vascular-compression-of-the-airway-in-patients-with-congenitalheart-disease-and-persistent-lower-respiratory-symptoms
#2
Tuğba Şişmanlar Eyüboğlu, Ayşe Tana Aslan, Çiğdem Öztunalı, Sedef Tunaoğlu, Ayşe Deniz Oğuz, Serdar Kula, Betül Derinkuyu, Öznur Boyunağa
Background/aim: Airway compression (AC) by vascular structures is an important complication of congenital heart disease (CHD) that often goes unrecognized. It is not easy to identify whether CHD patients require additional invasive examinations or not. Therefore, the present study aims to develop an AC diagnostic algorithm for CHD patients. Materials and methods: CHD patients with persistent respiratory symptoms that were treated between January 2007 and December 2015 were retrospectively reviewed. The following data were recorded for all CHD patients with AC: age, cardiac anomalies, the compressed structure, the airway diameter ratio (ADR), the compressing structure(s), treatment, and follow-up...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29147800/the-pediatric-heart-failure-workforce-an-international-multicenter-survey
#3
Scott R Auerbach, Melanie D Everitt, Ryan J Butts, David N Rosenthal, Yuk M Law
Our objective was to understand the scope of pediatric heart failure (HF) and the current staffing environment of HF programs. An online survey was distributed to members of the Pediatric Heart Transplant Study and the Pediatric Council of the International Society for Heart and Lung Transplantation. All participants received the primary 23-question survey. Additionally, HF program directors received a 32-question supplemental survey. Of 235 invitations sent, there were 69 (29%) primary surveys and 34 program director surveys completed (24 U...
November 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29147786/risk-factors-for-development-of-ectopic-atrial-tachycardia-in-post-operative-congenital-heart-disease
#4
Bradley C Clark, John T Berger, Charles I Berul, Richard A Jonas, Jonathan R Kaltman, Julianne Lapsa, Dilip S Nath, Elizabeth D Sherwin, Pranava Sinha, David Zurakowski, Jeffrey P Moak
Ectopic atrial tachycardia (EAT) is common in surgically repaired congenital heart disease (CHD) and carries the potential for significant hemodynamic compromise. Our objective was to determine the incidence, and risk factors of EAT after CHD surgery. Prospective study of patients that underwent surgery for CHD from February to October 2016 was performed. Demographic, perioperative and electrophysiologic data were collected. Sustained EAT (> 30 s) was documented by telemetry or electrocardiogram and confirmed by a pediatric electrophysiologist...
November 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29131462/outcomes-of-liver-transplantation-in-pediatric-recipients-with-cardiovascular-disease
#5
Márcio Miranda Brito, Joao Seda Neto, Eduardo A Fonseca, Renata Pugliese, Vera B Danesi, Helry L Candido, Adriana Porta, Cristian V B Borges, Gilda Porta, Paulo Chapchap, Irene Kazue Miura
LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD...
November 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29128521/prenatal-chromosomal-microarray-analysis-in-fetuses-with-congenital-heart-disease-a-prospective-cohort-study
#6
Yan Wang, Li Cao, Dong Liang, Lulu Meng, Yun Wu, Fengchang Qiao, Xiuqing Ji, Chunyu Luo, Jingjing Zhang, Tianhui Xu, Bin Yu, Leilei Wang, Ting Wang, Qiong Pan, Dingyuan Ma, Ping Hu, Zhengfeng Xu
BACKGROUD: Currently, chromosomal microarray analysis is considered as the first-tier test in pediatric care and prenatal diagnosis. However, the diagnostic yield of CMA for prenatal diagnosis of congenital heart disease has not been evaluated based on a large cohort. OBJECTIVE: Our aim was to evaluate the clinical utility of chromosomal microarray as the first-tier test for chromosomal abnormalities in fetuses with congenital heart disease. STUDY DESIGN: In this prospective study, 602 prenatal cases of congenital heart disease were investigated using SNP array over a 5-year period...
November 8, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29126909/-demographic-analysis-of-a-congenital-heart-disease-clinic-of-the-mexican-institute-of-social-security-with-special-interest-in-the-adult
#7
Horacio Márquez-González, Lucelli Yáñez-Gutiérrez, Jimena Lucely Rivera-May, Diana López-Gallegos, Eduardo Almeida-Gutiérrez
INTRODUCTION: Congenital heart disease (CHD) has an incidence of 8-10 cases per 1000 live births. In Mexico, there are 18,000-20,000 new cases per year. Most tertiary care centers for CHD attend only pediatric population; the Mexican Institute of Social Security (IMSS) has a clinic that attends pediatric and adult population. OBJECTIVE: To analyze the demographic aspects of the CHD clinic of IMSS. METHODS: From 2011 to 2016 a cross-sectional study of the CC clinic of a third level hospital of the IMSS, including all patients with confirmed structural heart disease of recent diagnosis was carried out...
November 7, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/29124558/development-of-a-simple-device-enabling-percutaneous-flow-regulation-for-a-small-vascular-graft-for-a-blalock-taussig-shunt-capable-of-flow-regulation-complete-translation-of-a-review-article-originally-published-in-pediatric-cardiology-and-cardiac-surgery
#8
Yoshikazu Motohashi, Ryo Shimada, Tomoyasu Sasaki, Takahiro Katsumata, Kazunori Dan, Yasuhiro Tsutsui, Shintaro Nemoto
OBJECTIVES: The Blalock-Taussig shunt (BTS) operation is a cornerstone as initial palliative surgery for congenital heart disease with severely reduced pulmonary blood flow (PBF). The ideal PBF provided by BTS is crucial for an uneventful postoperative course, since excess PBF results in acute distress of the systemic circulation and insufficient PBF requires another BTS surgery. Therefore, the goal of this study was to develop a simple device to control the shunt graft flow percutaneously using a constrictor balloon connected to a subcutaneous port...
November 9, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29107545/infectious-complications-of-ventricular-assist-device-use-in-children-in-the-united-states-data-from-the-pediatric-interagency-registry-for-mechanical-circulatory-support-pedimacs
#9
Scott R Auerbach, Marc E Richmond, Kurt R Schumacher, Dalia Lopez-Colon, Max B Mitchell, Mark W Turrentine, Ryan S Cantor, Robert A Niebler, Pirooz Eghtesady
BACKGROUND: Infections are frequent in pediatric ventricular assist device (VAD) patients. In this study we aimed to describe infections in durable VAD patients reported to Pedimacs. METHODS: Durable VAD data from the Pedimacs registry (September 19, 2012 to December 31, 2015) were analyzed. Infections were described with standard descriptive statistics, Kaplan-Meier analysis and competing outcomes analysis. RESULTS: There were 248 implants in 222 patients, with a mean age and a median follow-up of 11 ± 6...
October 3, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29106863/clinical-variables-determining-the-success-of-adenotonsillectomy-in-children-with-down-syndrome
#10
Maíra da Rocha, Renata Caroline Mendonça Ferraz, Vitor Guo Chen, Gustavo Antonio Moreira, Reginaldo Raimundo Fujita
OBJECTIVES: To evaluate the evolution of polysomnographic parameters of children with Down syndrome and obstructive sleep apnea syndrome submitted to adenotonsillectomy and the interaction of comorbidities on therapeutic outcome. METHODS: Ninety patients with Down syndrome and habitual snoring were identified between 2005 and 2015 in a Pediatric Otorhinolaryngology Clinic. Parent's complaints were evaluated by the test of equality of two proportions. Wilcoxon test was used to examine pre- and post-operative polysomnographic differences...
November 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29100090/exome-wide-association-study-identifies-greb1l-mutations-in-congenital-kidney-malformations
#11
Simone Sanna-Cherchi, Kamal Khan, Rik Westland, Priya Krithivasan, Lorraine Fievet, Hila Milo Rasouly, Iuliana Ionita-Laza, Valentina P Capone, David A Fasel, Krzysztof Kiryluk, Sitharthan Kamalakaran, Monica Bodria, Edgar A Otto, Matthew G Sampson, Christopher E Gillies, Virginia Vega-Warner, Katarina Vukojevic, Igor Pediaditakis, Gabriel S Makar, Adele Mitrotti, Miguel Verbitsky, Jeremiah Martino, Qingxue Liu, Young-Ji Na, Vinicio Goj, Gianluigi Ardissino, Maddalena Gigante, Loreto Gesualdo, Magdalena Janezcko, Marcin Zaniew, Cathy Lee Mendelsohn, Shirlee Shril, Friedhelm Hildebrandt, Joanna A E van Wijk, Adela Arapovic, Marijan Saraga, Landino Allegri, Claudia Izzi, Francesco Scolari, Velibor Tasic, Gian Marco Ghiggeri, Anna Latos-Bielenska, Anna-Materna Kiryluk, Shrikant Mane, David B Goldstein, Richard P Lifton, Nicholas Katsanis, Erica E Davis, Ali G Gharavi
Renal agenesis and hypodysplasia (RHD) are major causes of pediatric chronic kidney disease and are highly genetically heterogeneous. We conducted whole-exome sequencing in 202 case subjects with RHD and identified diagnostic mutations in genes known to be associated with RHD in 7/202 case subjects. In an additional affected individual with RHD and a congenital heart defect, we found a homozygous loss-of-function (LOF) variant in SLIT3, recapitulating phenotypes reported with Slit3 inactivation in the mouse...
November 2, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29092770/avalon-catheters-in-pediatric-patients-requiring-ecmo-placement-and-migration-problems
#12
Marcus D Jarboe, Samir K Gadepalli, Joseph T Church, Meghan A Arnold, Ronald B Hirschl, George B Mychaliska
PURPOSE: The Avalon dual-lumen venovenous catheter has several advantages, but placement techniques and management have not been adequately addressed in the pediatric population. We assessed our institutional outcomes and complications using the Avalon catheter in children. METHODS: We reviewed all pediatric patients who had Avalon catheters placed for respiratory failure at our institution, excluding congenital heart disease patients, from April 2009 to March 2016...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29090255/anesthetic-considerations-for-a-pediatric-patient-with-wolf-hirschhorn-syndrome-a-case-report
#13
Masanori Tsukamoto, Hitoshi Yamanaka, Takeshi Yokoyama
Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management...
September 2017: J Dent Anesth Pain Med
https://www.readbyqxmd.com/read/29079892/the-genetic-counselor-in-the-pediatric-arrhythmia-clinic-review-and-assessment-of-services
#14
Benjamin M Helm, Samantha L Freeze, Katherine G Spoonamore, Stephanie M Ware, Mark D Ayers, Adam C Kean
There are minimal data on the impact of genetic counselors in subspecialty clinics, including the pediatric arrhythmia clinic. This study aimed to describe the clinical encounters of a genetic counselor integrated into a pediatric arrhythmia clinic. In the 20 months between July 2015 and February 2017, a total of 1914 scheduled patients were screened for indications relevant for assessment by a genetic counselor. Of these, the genetic counselor completed 276 patient encounters, seeing 14.4% of all patients in clinic...
October 27, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/29076654/the-respiratory-system-in-pediatric-chronic-heart-disease
#15
REVIEW
Sotiria C Apostolopoulou
Cardiovascular disease in the pediatric population closely affects the respiratory system inducing water retention in the lungs and pulmonary edema, airway compression by cardiovascular structures, restrictive pulmonary physiology as a result of hemodynamic changes and surgical repair, susceptibility to respiratory infections, development of pulmonary hypertension, thrombosis, or hemorrhage. Chronic heart failure and congenital heart disease are characterized by various respiratory manifestations and symptoms mimicking lung disease, which are frequently difficult to diagnose and treat...
December 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29071790/stress-and-quality-of-life-among-parents-of-children-with-congenital-heart-disease-referred-for-psychological-services
#16
Astrida Kaugars, Clarissa Shields, Cheryl Brosig
OBJECTIVE: The study examined parent stress and health-related quality of life (HRQOL) among families of children with congenital heart disease (CHD) referred for psychological services. METHODS: Parents of 54 children (85% boys) aged 3 to 13 (Mage  = 7.48, SD = 2.38) completed measures to assess parenting stress (Parenting Stress Index - Short Form; Pediatric Inventory for Parents) and the PedsQL Family Impact Module. Medical information was retrieved from medical record review...
October 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29068106/clinical-profile-of-congenital-rubella-syndrome-in-yogyakarta-indonesia
#17
Elisabeth S Herini, Gunadi, Agung Triono, Fita Wirastuti, Kristy Iskandar, Niprida Mardin, Yati Soenarto
BACKGROUND: Congenital rubella syndrome (CRS) has many severe neurological manifestations and other systemic consequences in patients. Although various studies have been done in Indonesia, there have not been any conclusive results on CRS incidence. Therefore, we aimed to investigate the incidence, clinical manifestations and outcomes of CRS in Yogyakarta, Indonesia. METHODS: A descriptive study involving a review of congenital anomalies associated with CRS cases at Dr...
October 25, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29063953/magnetic-resonance-myocardial-perfusion-imaging-safety-and-indications-in-pediatrics-and-young-adults
#18
David M Biko, R Thomas Collins, Sara L Partington, Matthew Harris, Kevin K Whitehead, Marc S Keller, Mark A Fogel
The purpose of this study was to assess the safety and indications for cardiac magnetic resonance (CMR) with myocardial perfusion imaging (MPI) in a cohort of children and young adults. A retrospective review of 178 children and young adults who underwent CMR with MPI was performed. Studies were categorized based on study protocols as MPI with resting perfusion only, adenosine stress MPI, exercise-induced stress MPI, and MPI for cardiac mass diagnosis. Relevant clinical history, exam indications, and adverse reactions following gadolinium-based contrast agent and adenosine administration were recorded...
October 24, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29055602/late-graft-dysfunction-after-pediatric-heart-transplantation-is-associated-with-fibrosis-and-microvasculopathy-by-automated-digital-whole-slide-analysis
#19
Brian Feingold, Jennifer Picarsic, Andrew Lesniak, Benjamin A Popp, Michelle A Wood-Trageser, Anthony J Demetris
BACKGROUND: Histopathologic features of late graft dysfunction (LGD) in endomyocardial biopsies (EMBs) after pediatric heart transplantation (HT) have been incompletely described and rarely quantified. We employed automated, morphometric analysis of whole-slide EMB images to objectively quantify fibrosis and microvasculopathy after pediatric HT. METHODS: Nine recipients with clinical LGD were matched with controls on age, listing diagnosis, crossmatch and time since HT...
September 29, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29050751/fibrosis-related-gene-expression-in-single-ventricle-heart-disease
#20
Stephanie J Nakano, Austine K Siomos, Anastacia M Garcia, Hieu Nguyen, Megan SooHoo, Csaba Galambos, Karin Nunley, Brian L Stauffer, Carmen C Sucharov, Shelley D Miyamoto
OBJECTIVE: To evaluate fibrosis and fibrosis-related gene expression in the myocardium of pediatric subjects with single ventricle with right ventricular failure. STUDY DESIGN: Real-time quantitative polymerase chain reaction was performed on explanted right ventricular myocardium of pediatric subjects with single ventricle disease and controls with nonfailing heart disease. Subjects were divided into 3 groups: single ventricle failing (right ventricular failure before or after stage I palliation), single ventricle nonfailing (infants listed for primary transplantation with normal right ventricular function), and stage III (Fontan or right ventricular failure after stage III)...
October 16, 2017: Journal of Pediatrics
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