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"Congenital heart disease"

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https://www.readbyqxmd.com/read/29222866/outcome-of-antibody-mediated-rejection-compared-to-acute-cellular-rejection-after-pediatric-heart-transplantation
#1
Gabrielle R Vaughn, Neal W Jorgensen, Yuk M Law, Erin L Albers, Borah J Hong, Joshua M Friedland-Little, Mariska S Kemna
Outcomes of ACR after pediatric HTx have been well described, but less has been reported on outcomes of AMR. We compared the clinical characteristics and cardiovascular outcomes (composite end-point of death, retransplantation, or allograft vasculopathy) of pediatric HTx recipients with AMR, ACR, and no rejection in a retrospective single-center study of 104 recipients. Twenty were treated for AMR; 15 were treated for ACR. Recipients with AMR had an increased frequency of congenital heart disease (90% vs ACR 67% vs no rejection 59%, P = ...
December 9, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29222009/redefining-the-phenotypic-spectrum-of-de-novo-heterozygous-cdk13-variants-three-patients-without-cardiac-defects
#2
Tomoko Uehara, Toshiki Takenouchi, Rika Kosaki, Kenji Kurosawa, Seiji Mizuno, Kenjiro Kosaki
Recently, 7 patients with de novo constitutional non-synonymous mutations in the CDK13 gene were ascertained through a trio exome analysis of a large cohort of 610 patients with congenital cardiac diseases. Despite another report describing 9 additional patients, the clinical spectrum of this condition has yet to be defined. Herein, we report 3 patients with heterozygous constitutional CDK13 mutations, who were ascertained through exome analysis of children with intellectual disability and minor anomalies, who lacked cardiac anomalies...
December 5, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29221746/clinical-and-neuroimaging-characteristics-of-cerebral-sinovenous-thrombosis-in-neonates-undergoing-cardiac-surgery
#3
Nathalie H P Claessens, Selma O Algra, Nicolaas J G Jansen, Floris Groenendaal, Esther de Wit, Alexander A Wilbrink, Felix Haas, Antonius N J Schouten, Rutger A J Nievelstein, Manon J N L Benders, Linda S de Vries
OBJECTIVES: Neonates with congenital heart disease may have an increased risk of cerebral sinovenous thrombosis, but incidence rates are lacking. This study describes the clinical and neuroimaging characteristics of cerebral sinovenous thrombosis in neonates undergoing cardiac surgery. METHODS: Forty neonates (78% male) requiring neonatal univentricular or biventricular cardiac repair using cardiopulmonary bypass were included. All underwent preoperative (median postnatal day 7) and postoperative (median postoperative day 7) magnetic resonance imaging of the brain, including venography, to detect cerebral sinovenous thrombosis...
November 8, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29221691/characteristics-and-outcomes-of-pediatric-heart-failure-related-emergency-department-visits-in-the-united-states-a-population-based-study
#4
Erika J Mejia, Matthew J O'Connor, Kimberly Y Lin, Lihai Song, Heather Griffis, Christopher E Mascio, Pirouz Shamszad, Aaron Donoghue, Chitra Ravishankar, Robert E Shaddy, Joseph W Rossano
OBJECTIVES: To describe the frequency, characteristics, and outcomes of heart failure-related emergency department (ED) visits in pediatric patients. We aimed to test the hypothesis that these visits are associated with higher admission rates, mortality, and resource utilization. STUDY DESIGN: A retrospective analysis of the Nationwide Emergency Department Sample for 2010 of patients ≤18 years of age was performed to describe ED visits with and without heart failure...
December 6, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29219889/the-value-of-neutrophil-to-lymphocyte-ratio-and-platelet-to-lymphocyte-ratio-for-detecting-early-onset-neonatal-sepsis
#5
Emrah Can, Şahin Hamilcikan, Ceren Can
OBJECTIVE: The purpose of this study was to investigate the relationship between neonate early-onset sepsis (EOS) and the neutrophil to lymphocyte ratio (NLR) and the platelet to lymphocyte ratio (PLR) of term neonates. MATERIALS AND METHODS: This prospective observational study was conducted with term neonates diagnosed with EOS compared with 44 healthy controls. Exclusion criteria were prematurity, postmaturity, small or large for gestational age according to week of pregnancy, preeclampsia, gestational diabetes mellitus, chorioamnionitis, congenital major anomalies, and cyanotic congenital heart disease...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29218069/down-syndrome-genetics-and-cardiogenetics
#6
Vasilica Plaiasu
During the last years, Down syndrome has been the focus of special attention. Down syndrome is a genetic disorder characterized by distinct physical features and some degree of cognitive disability. Patients with Down syndrome also present many other congenital anomalies. The mapping for phenotypes to specific regions of chromosome 21 permits to identify which genes (or small regions) contribute to the phenotypic features of Down syndrome and thus, to understand its pathogenesis. Mainly there are three cytogenetic forms of Down syndrome: free trisomy 21, mosaic trisomy 21 and robertsonian translocation trisomy 21...
September 2017: Mædica
https://www.readbyqxmd.com/read/29217153/patterns-of-transition-experience-for-parents-going-home-from-hospital-with-their-infant-after-first-stage-surgery-for-complex-congenital-heart-disease
#7
Kerry L Gaskin
PURPOSE: The purpose of this study was to explore parents' experiences of one specific timepoint in their infant's journey: the transition from hospital to home, following the first stage of their infant's cardiac surgery for complex congenital heart disease. DESIGN AND METHODS: A prospective longitudinal mixed methods study, underpinned with Middle Range Transition Theory (Meleis, Sawyer, Im, Hilfinger Messias, & Schumacher, 2000). Face to face and telephone interviews were conducted and self-report forms completed by parents at four-time points: before discharge (T0), 2weeks after discharge (T1), 8weeks after discharge (T2) and after stage two surgery (T3)...
December 4, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/29216946/-diagnostic-significance-of-urinary-neutrophil-gelatin-enzyme-related-lipid-delivery-protein-and-kidney-injury-molecule-1-in-acute-kidney-injury-after-cardiac-operation-with-cardiopulmonary-bypass-operation-in-children
#8
Yupeng Wen, Zongxiao Li, Cheng Chang, Peng Zhang, Yang Lyu
OBJECTIVE: To investigate the diagnostic value of urinary neutrophil gelatin enzyme-related lipid delivery protein (NGAL) and kidney injury molecule-1 (KIM-1) in the acute kidney injury (AKI) after cardiopulmonary bypass (CPB) operation in children with non-cyanotic congenital heart disease (CHD). METHODS: A retrospective analysis was conducted. 200 CPB undergoing cardiac surgery in children with non-cyanotic CHD admitted to Tianjin Children's Hospital from June 2015 to May 2017 were enrolled...
December 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/29214893/educational-dvd-for-parents-of-children-with-congenital-heart-disease-a-pilot-study
#9
Derrick H Z Ha, Margaret Hay, Samuel Menahem
Parental knowledge regarding their child's congenital heart defect (CHD) is limited. This study developed and evaluated an interactive DVD that helped parents to understand their child's CHD. A DVD describing four cardiac abnormalities was created using videos and Adobe Flash. The parents' knowledge was assessed via a questionnaire before and after receiving the DVD. A control group did not receive the DVD. The DVDs were successfully created and improved the parental knowledge of their child's cardiac anomaly...
December 7, 2017: Journal of Visual Communication in Medicine
https://www.readbyqxmd.com/read/29214485/two-phase-contrast-injection-protocol-for-pediatric-cardiac-computed-tomography-in-children-with-congenital-heart-disease
#10
Naoki Fukuyama, Akira Kurata, Naoto Kawaguchi, Ryo Tashiro, Takashi Higaki, Takahiro Yokoi, Yuki Tanabe, Hikaru Nishiyama, Toshihide Itoh, Teruhito Kido, Masao Miyagawa, Teruhito Mochizuki
To assess a two-phase contrast injection protocol for contrast enhancement during cardiac computed tomography (CT) in children with congenital heart disease. Forty-three children (20 boys, 23 girls) of median age 13 months (range 3 days-8.3 years) and weighing ≤ 20 kg who underwent cardiac CT using a two-phase contrast injection protocol at our institution were retrospectively identified. High-pitch spiral third-generation dual-source cardiac CT (tube voltage 70 kV) was performed with a fixed delay of 60 s after contrast injection in the order of 10 mgI/kg/s (30 s), 15 mgI/kg/s (20 s), and a saline chaser (10 s)...
December 6, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29214238/redox-regulation-of-a-gain-of-function-mutation-n308d-in-shp2-noonan-syndrome
#11
Luciana E S F Machado, David A Critton, Rebecca Page, Wolfgang Peti
SHP2 (Src homology 2 domain-containing protein tyrosine phosphatase 2; PTPN11) is a ubiquitous multidomain, nonreceptor protein tyrosine phosphatase (PTP) that plays an important role in diseases such as cancer, diabetes, and Noonan syndrome (NS). NS is one of the most common genetic disorders associated with congenital heart disease, and approximately half of the patients with Noonan syndrome have gain-of-function mutations in SHP2. One of the most common NS mutations is N308D. The activity of SHP2, like that of most PTPs, is reversibly inactivated by reactive oxygen species (ROS)...
November 30, 2017: ACS Omega
https://www.readbyqxmd.com/read/29212650/low-nasal-no-in-congenital-heart-disease-with-systemic-right-ventricle-and-postcardiac-transplantation
#12
Phillip S Adams, Maliha Zahid, Omar Khalifa, Brian Feingold, Cecilia W Lo
BACKGROUND: NO bioavailability has not been systematically examined in congenital heart disease (CHD). To assess NO in patients with CHD, we measured nasal NO (nNO) generated by the nasal epithelia, given blood NO is difficult to measure (half-life, <2 ms). Given NO's role in hemodynamic regulation and the association of NO bioavailability with heart failure risk, we hypothesized NO levels may differ with varying severity of CHD physiologic characteristics. METHODS AND RESULTS: Six-hundred eighteen subjects, 483 with CHD and 135 controls, had nNO measured noninvasively via the nares using American Thoracic Society/European Respiratory Society guidelines...
December 6, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29211855/a-rabbit-model-of-progressive-chronic-right-ventricular-pressure-overload
#13
Sara Roldan Ramos, Guido Pieles, Wei Hui, Cameron Slorach, Andrew N Redington, Mark K Friedberg
OBJECTIVES: Right ventricular (RV) failure from increased pressure loading is a frequent consequence of acquired and congenital heart diseases. However, the mechanisms involved in their pathophysiology are still unclear, and few data exist on RV pressure-loading models and early versus late effects on RV and left ventricular responses. We characterized a rabbit model of chronic RV pressure overload and early-late effects on biventricular function. METHODS: Twenty-one New Zealand white rabbits were randomized into 3 groups: (i) sham, (ii) pulmonary artery (PA) banding (PAB) for 3 weeks (PAB3W) and (iii) PAB for 6 weeks (PAB6W)...
December 2, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29211219/incidence-and-treatment-of-chylothorax-in-children-undergoing-corrective-surgery-for-congenital-heart-diseases
#14
Nicolle Martin Christofe, Cristiane Felix Ximenes Pessotti, Laércio Paiva, Ieda Biscegli Jatene
INTRODUCTION: Chylothorax is a lymphatic extravasation into pleural cavity and its incidence is 0.25%-5.3% in children undergoing cardiac surgery. OBJECTIVE: To evaluate the incidence of chylothorax in pediatrics patients operated, linking it in each surgical intervention. Evaluate treatment types and efficiency. METHODS: Retrospective study using medical records of children undergoing cardiac surgery in the Hospital do Coração between 2004 and 2014...
September 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29211217/application-of-unilateral-pectoralis-major-muscle-flap-in-the-treatment-of-sternal-wound-dehiscence
#15
Grazielle de Souza Horácio, Pedro Soler Coltro, Antonio Albacete, Juliano Baron Almeida, Vinícius Zolezi da Silva, Ivan de Rezende Almeida, Alfredo José Rodrigues, Jayme Adriano Farina
OBJECTIVE: This study aims to report the use of the unilateral pectoralis major muscle flap for the treatment of the sternal wound dehiscence. METHODS: A retrospective study including patients who underwent unilateral pectoralis major muscle flap was performed for the treatment of sternotomy dehiscence due to coronary artery bypass, valve replacement, congenital heart disease correction and mediastinitis, between 1997 and 2016. Data from the epidemiological profile of patients, length of hospital stay, postoperative complications and mortality rate were obtained...
September 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29210924/intraventricular-hemorrhage-in-moderate-to-severe-congenital-heart-disease
#16
Cynthia M Ortinau, Jagruti S Anadkat, Christopher D Smyser, Pirooz Eghtesady
OBJECTIVES: Determine the prevalence of intraventricular hemorrhage in infants with moderate to severe congenital heart disease, investigate the impact of gestational age, cardiac diagnosis, and cardiac intervention on intraventricular hemorrhage, and compare intraventricular hemorrhage rates in preterm infants with and without congenital heart disease. DESIGN: A single-center retrospective review. SETTING: A tertiary care children's hospital...
November 28, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29210771/effect-of-inhaled-nitric-oxide-on-hemodynamics-in-lambs-with-1%C3%A2-ventricle-circulation
#17
Hitoshi Kanamitsu, Yasuhiro Fujii, Luca Centola, Katsushi Kinouchi, Liqun Zhu, Robert K Riemer, Olaf Reinhartz
Inhaled nitric oxide (NO) is widely used to treat postoperative pulmonary hypertension in congenital heart disease. It is believed that NO increases cardiac output (CO) by decreasing pulmonary vascular resistance (PVR), leading to increased left ventricular preload. However, the effect of NO on CO in patients with 1½ ventricle circulation remains unclear. To evaluate this, a superior cavopulmonary (SCP) shunt was constructed in 10 juvenile sheep. A PTFE graft was inserted between the superior vena cava (SVC) and the main pulmonary artery (PA)...
November 27, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29209720/association-of-us-state-implementation-of-newborn-screening-policies-for-critical-congenital-heart-disease-with-early-infant-cardiac-deaths
#18
Rahi Abouk, Scott D Grosse, Elizabeth C Ailes, Matthew E Oster
Importance: In 2011, critical congenital heart disease was added to the US Recommended Uniform Screening Panel for newborns, but whether state implementation of screening policies has been associated with infant death rates is unknown. Objective: To assess whether there was an association between implementation of state newborn screening policies for critical congenital heart disease and infant death rates. Design, Setting, and Participants: Observational study with group-level analyses...
December 5, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29209703/the-success-of-state-newborn-screening-policies-for-critical-congenital-heart-disease
#19
Alex R Kemper, Wendy K K Lam, Joseph A Bocchini
No abstract text is available yet for this article.
December 5, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29209541/a-rare-manifestation-of-asymptomatic-ebstein-s-anomaly-with-tricuspid-valve-endocarditis
#20
Carmel Moazez, Vicken Zeitjian, Christian Breburda, Ranjini Roy
Ebstein's anomaly is a rare congenital heart disease that presents with apical displacement of the septal and posterior leaflets of the tricuspid valve. It has a wide spectrum of clinical presentations and has been shown to manifest itself any time from birth to adulthood. Our patient is a 43-year-old male with a history of intravenous heroin abuse who presented to the emergency department with worsening shortness of breath and lower extremity edema. He denied any prior cardiac history. A transthoracic echo showed normal left ventricular function, but a large 2...
2017: Case Reports in Cardiology
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