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pediatric congenital heart diseases

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https://www.readbyqxmd.com/read/28746169/traditional-long-term-central-venous-catheters-versus-transhepatic-venous-catheters-in-infants-and-young-children
#1
Amanda Marie Marshall, David A Danford, Christopher L Curzon, Venus Anderson, Jeffrey W Delaney
OBJECTIVES: Children with congenital heart disease may require long-term central venous access for intensive care management; however, central venous access must also be preserved for future surgical and catheterization procedures. Transhepatic venous catheters may be an useful alternative. The objective of this study was to compare transhepatic venous catheters with traditional central venous catheters regarding complication rate and duration of catheter service. DESIGN: Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters...
July 25, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28736822/congenital-heart-disease-and-cardiac-procedural-outcomes-in-patients-with-trisomy-21-and-turner-syndrome
#2
Raysa Morales-Demori
Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%-60%), atrial (16%-21%), or ventricular septal defects (14%-27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions...
July 24, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28731912/assessing-infant-and-maternal-readiness-for-newborn-discharge
#3
Ling Jing, Casidhe-Nicole Bethancourt, Thomas McDonagh
PURPOSE OF REVIEW: The review highlights the shift from prescribed length of stay (LOS) to mother-infant dyad readiness as the basis for making discharge decisions for healthy term newborns. We describe the components of readiness that should be considered in making the decision, focusing on infant clinical readiness, and maternal and familial readiness. RECENT FINDINGS: Although the Newborns' and Mothers' Health Protection Act of 1996 aimed to protect infants and mothers by establishing a minimum LOS, the American Academy of Pediatrics 2015 policy on newborn discharge acknowledges the shift from LOS-based to readiness-based discharge decision-making...
July 20, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28729908/aki-after-pediatric-cardiac-surgery-for-congenital-heart-diseases-recent-developments-in-diagnostic-criteria-and-early-diagnosis-by-biomarkers
#4
REVIEW
Yuichiro Toda, Kentaro Sugimoto
BACKGROUND: Acute kidney injury (AKI) after cardiac surgery in children with congenital heart disease is a common complication. AKI is also associated with high morbidity and mortality. The Kidney Diseases Improving Global Outcomes (KDIGO) criteria for AKI classification are now widely used for the definition of AKI. It is noteworthy that a statement about children was added to the criteria. Many studies aimed at finding useful biomarkers are now being performed by using these criteria...
2017: Journal of Intensive Care
https://www.readbyqxmd.com/read/28719142/changing-prevalence-of-severe-congenital-heart-disease-results-from-the-national-register-for-congenital-heart-defects-in-germany
#5
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/28711963/transfer-of-neonates-with-critical-congenital-heart-disease-within-a-regionalized-network
#6
Michael F Swartz, Jill M Cholette, Jennifer M Orie, Marshall L Jacobs, Jeffrey P Jacobs, George M Alfieris
Regionalization of pediatric cardiac surgical care varies between and within states. In most geographic regions, at least some neonates with critical heart disease are transferred from their birth hospital to a different hospital for surgery. The impact of neonatal transfer for surgery, particularly over a considerable distance (>10 miles), has been largely unexplored. We sought to examine the impact of transferring neonates for cardiac surgery. We queried the New York State Cardiac Surgery database (2005-2014) from a single institution to identify neonates born within the cardiac surgery center and those transferred for surgery...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28705803/clinical-practice-guidelines-for-the-care-of-girls-and-women-with-turner-syndrome-proceedings-from-the-2016-cincinnati-international-turner-syndrome-meeting
#7
Claus H Gravholt, Niels H Andersen, Gerard S Conway, Olaf M Dekkers, Mitchell E Geffner, Karen O Klein, Angela E Lin, Nelly Mauras, Charmian A Quigley, Karen Rubin, David E Sandberg, Theo C J Sas, Michael Silberbach, Viveca Söderström-Anttila, Kirstine Stochholm, Janielle A van Alfen-van derVelden, Joachim Woelfle, Philippe F Backeljauw
Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016...
September 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28704916/three-dimensional-echocardiography-in-adult-congenital-heart-disease
#8
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/28702947/initial-validation-of-a-healthcare-needs-scale-for-youth-with-congenital-heart-disease
#9
Chi-Wen Chen, Ciao-Lin Ho, Wen-Jen Su, Jou-Kou Wang, Hung-Tao Chung, Pi-Chang Lee, Chun-Wei Lu, Be-Tau Hwang
AIM: To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. BACKGROUND: As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. DESIGN: A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease...
July 12, 2017: Journal of Advanced Nursing
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#10
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28701597/randomized-comparative-study-of-intravenous-infusion-of-three-different-fixed-doses-of-milrinone-in-pediatric-patients-with-pulmonary-hypertension-undergoing-open-heart-surgery
#11
Neeraj Kumar Barnwal, Sanjeeta Rajendra Umbarkar, Manjula Sudeep Sarkar, Raylene J Dias
BACKGROUND: Pulmonary hypertension secondary to congenital heart disease is a common problem in pediatric patients presenting for open heart surgery. Milrinone has been shown to reduce pulmonary vascular resistance and pulmonary artery pressure in pediatric patients and neonates postcardiac surgery. We aimed to evaluate the postoperative outcome in such patients with three different fixed maintenance doses of milrinone. METHODOLOGY: Patients were randomized into three groups...
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28699323/intra-procedural-continuous-dialysis-to-facilitate-interventional-catheterization-in-pediatric-patients-with-severe-renal-failure
#12
Angeline D Opina, Athar M Qureshi, Eileen Brewer, Ewa Elenberg, Sarah Swartz, Mini Michael, Henri Justino
BACKGROUND: Interventional catheterization procedures may be needed for patients with severe renal failure who are dependent on dialysis. To avoid the risk of fluid overload and electrolyte derangement during complex procedures in this oliguric/anuric patient population, we performed intra-procedural dialysis, either continuous renal replacement therapy (CRRT) or continous cycling peritoneal dialysis (CCPD). METHODS: We performed a retrospective review of a cohort of pediatric patients, ages 0-18 years, with dialysis-dependent renal failure who received CRRT or CCPD during catheterization procedures from January 2013 to March 2016...
July 12, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28697825/-screening-and-follow-up-for-congenital-heart-disease-in-children-aged-0-3-years-in-rural-areas-of-chongqing-china
#13
Lei Zhang, Mei-Yu An, Bing Zhu, Wan-Dong Shen, Shu-Jiang Tan, Xiao-Juan Ji, Jie Tian, Xiao-Yan Liu
OBJECTIVE: To examine the incidence of congenital heart disease (CHD) in children aged 0-3 years in the rural areas of Chongqing, and to determine the suitable "screening-diagnosis-follow-up" system and screening indicators for CHD in these areas. METHODS: Children aged 0-3 years from rural areas of the Fuling Disctrict of Chongqing were selected by cluster sampling. Using the "screening-diagnosis-evaluation system" employed at the levels of village/town, district/county, and province/city, the children were screened for seven indicators, i...
July 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28696882/risk-factors-for-mortality-in-reoperations-for-pediatric-and-congenital-heart-surgery-in-a-developing-country
#14
Carlos A Villa-Hincapie, Marisol Carreno-Jaimes, Carlos E Obando-Lopez, Jaime Camacho-Mackenzie, Juan P Umaña-Mallarino, Nestor F Sandoval-Reyes
BACKGROUND: The survival of patients with congenital heart disease has increased in the recent years, because of enhanced diagnostic capabilities, better surgical techniques, and improved perioperative care. Many patients will require reoperation as part of staged procedures or to treat grafts deterioration and residual or recurrent lesions. Reoperations favor the formation of cardiac adhesions and consequently increase surgery time; however, the impact on morbidity and operative mortality is certain...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28696875/status-and-challenges-of-care-in-africa-for-adults-with-congenital-heart-defects
#15
Frank Edwin, Liesl Zühlke, Heba Farouk, Ana Olga Mocumbi, Kow Entsua-Mensah, Desrie Delsol-Gyan, Fidelia Bode-Thomas, Andre Brooks, Blanche Cupido, Mark Tettey, Ernest Aniteye, Martin M Tamatey, Kofi B Gyan, Jacques Cabral Tantchou Tchoumi, Mohamed-Adel Elgamal
The 54 countries in Africa have an estimated total annual congenital heart defect (CHD) birth prevalence of 300,486 cases. More than half (51.4%) of the continental birth prevalence occurs in only seven countries. Congenital heart disease remains primarily a pediatric health issue in Africa because of the deficient health-care systems: the adults with CHD made up just 10% of patients with CHD in Ghana, and 13.7% of patients with CHD presenting for surgery in Mozambique. With Africa's population projected to double in the next 35 years, the already deficient health systems for CHD care will suffer unbearable strain unless determined and courageous action is undertaken by the African leaders...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28691936/national-variation-in-the-use-of-tracheostomy-in-patients-with-congenital-heart-disease
#16
Joyce T Johnson, Bradley S Marino, Darren Klugman, Pirouz Shamszad
OBJECTIVES: The postsurgical care of children with congenital heart disease may be complicated by the need for cardiorespiratory support, including tracheostomy. The variation of the use of tracheostomy across multiple pediatric cardiac surgical centers has not been defined. We describe multicenter variation in the use of tracheostomy in children undergoing congenital heart surgery. DESIGN: We retrospectively analyzed a multicenter cohort. SETTING: Pediatric Health Information Systems database retrospective cohort...
July 6, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28688027/pharmacokinetics-of-fentanyl-and-its-derivatives-in-children-a-comprehensive-review
#17
REVIEW
Victoria C Ziesenitz, Janelle D Vaughns, Gilbert Koch, Gerd Mikus, Johannes N van den Anker
Fentanyl and its derivatives sufentanil, alfentanil, and remifentanil are potent opioids. A comprehensive review of the use of fentanyl and its derivatives in the pediatric population was performed using the National Library of Medicine PubMed. Studies were included if they contained original pharmacokinetic parameters or models using established routes of administration in patients younger than 18 years of age. Of 372 retrieved articles, 44 eligible pharmacokinetic studies contained data of 821 patients younger than 18 years of age, including more than 46 preterm infants, 64 full-term neonates, 115 infants/toddlers, 188 children, and 28 adolescents...
July 7, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28687891/functional-analyses-of-a-novel-cited2-nonsynonymous-mutation-in-chinese-tibetan-patients-with-congenital-heart-disease
#18
Shiming Liu, Zhaobing Su, Sainan Tan, Bin Ni, Hong Pan, Beihong Liu, Jing Wang, Jianmin Xiao, Qiuhong Chen
CITED2 gene is an important cardiac transcription factor that plays a fundamental role in the formation and development of embryonic cardiovascular. Previous studies have showed that knock-out of CITED2 in mice might result in various cardiac malformations. However, the mechanisms of CITED2 mutation on congenital heart disease (CHD) in Chinese Tibetan population are still poorly understood. In the present study, 187 unrelated Tibetan patients with CHD and 200 unrelated Tibetan healthy controls were screened for variants in the CITED2 gene; we subsequently identified one potential disease-causing mutation p...
July 8, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28687669/atrioventricular-nodal-reentrant-tachycardia-in-patients-with-congenital-heart-disease-outcome-after-catheter-ablation
#19
John Papagiannis, Daniel Joseph Beissel, Ulrich Krause, Michel Cabrera, Marta Telishevska, Stephen Seslar, Christopher Johnsrude, Charles Anderson, Svjetlana Tisma-Dupanovic, Diana Connelly, Dimosthenis Avramidis, Christopher Carter, Laszlo Kornyei, Ian Law, Nicholas Von Bergen, Jan Janusek, Jennifer Silva, Eric Rosenthal, Mark Willcox, Peter Kubus, Gabriele Hessling, Thomas Paul
BACKGROUND: The relationship of atrioventricular nodal reentrant tachycardia to congenital heart disease (CHD) and the outcome of catheter ablation in this population have not been studied adequately. METHODS AND RESULTS: A multicenter retrospective study was performed on patients with CHD who had atrioventricular nodal reentrant tachycardia and were treated with catheter ablation. There were 109 patients (61 women), aged 22.1±13.4 years. The majority, 86 of 109 (79%), had CHD resulting in right heart pressure or volume overload...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28683534/a-case-of-alagille-syndrome-presenting-with-chronic-cholestasis-in-an-adult
#20
Jihye Kim, Bumhee Yang, Namyoung Paik, Yon Ho Choe, Yong-Han Paik
Alagille syndrome (AGS) is a complex multisystem disorder that involves mainly the liver, heart, eyes, face, and skeleton. The main associated clinical features are chronic cholestasis due to a paucity of intrahepatic bile ducts, congenital heart disease primarily affecting pulmonary arteries, vertebral abnormalities, ocular embryotoxon, and peculiar facies. The manifestations generally become evident at a pediatric age. AGS is caused by defects in the Notch signaling pathway due to mutations in JAG1 or NOTCH2...
July 7, 2017: Clinical and Molecular Hepatology
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