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Heart disease in children

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https://www.readbyqxmd.com/read/29473337/acoustic-radiation-force-impulse-of-the-liver-after-fontan-operation-correlation-with-cardiopulmonary-exercise-test
#1
Michael V Di Maria, Lindsey Silverman, Adel K Younoszai, Mariana L Meyers, Kelley E Capocelli, Michael R Narkewicz
BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort...
February 23, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29472291/outcomes-after-surgical-coronary-artery-revascularisation-in-children-with-congenital-heart-disease
#2
Kalpana Thammineni, Jeffrey M Vinocur, Brian Harvey, Jeremiah S Menk, Michael Scott Kelleman, Anna-Maria Korakiti, Amanda S Thomas, James H Moller, James D St Louis, Lazaros K Kochilas
OBJECTIVE: Surgical coronary revascularisation in children with congenital heart disease (CHD) is a rare event for which limited information is available. In this study, we review the indications and outcomes of surgical coronary revascularisation from the Pediatric Cardiac Care Consortium, a large US-based multicentre registry of interventions for CHD. METHODS: This is a retrospective cohort study of children (<18 years old) with CHD who underwent surgical coronary revascularisation between 1982 and 2011...
February 22, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29471389/the-effect-of-paternal-factors-on-perinatal-and-paediatric-outcomes-a-systematic-review-and-meta-analysis
#3
Nan B Oldereid, Ulla-Britt Wennerholm, Anja Pinborg, Anne Loft, Hannele Laivuori, Max Petzold, Liv Bente Romundstad, Viveca Söderström-Anttila, Christina Bergh
BACKGROUND: Maternal factors, including increasing childbearing age and various life-style factors, are associated with poorer short- and long-term outcomes for children, whereas knowledge of paternal parameters is limited. Recently, increasing paternal age has been associated with adverse obstetric outcomes, birth defects, autism spectrum disorders and schizophrenia in children. OBJECTIVE AND RATIONALE: The aim of this systematic review is to describe the influence of paternal factors on adverse short- and long-term child outcomes...
February 15, 2018: Human Reproduction Update
https://www.readbyqxmd.com/read/29470938/diagnostic-performance-of-mr-elastography-for-liver-fibrosis-in-children-and-young-adults-with-a-spectrum-of-liver-diseases
#4
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
https://www.readbyqxmd.com/read/29470837/past-present-and-future-approaches-to-the-prevention-and-treatment-of-respiratory-syncytial-virus-infection-in-children
#5
REVIEW
Eric A F Simões, Louis Bont, Paolo Manzoni, Brigitte Fauroux, Bosco Paes, Josep Figueras-Aloy, Paul A Checchia, Xavier Carbonell-Estrany
INTRODUCTION: The REGAL (RSV Evidence - A Geographical Archive of the Literature) series has provided a comprehensive review of the published evidence in the field of respiratory syncytial virus (RSV) in Western countries over the last 20 years. This seventh and final publication covers the past, present and future approaches to the prevention and treatment of RSV infection among infants and children. METHODS: A systematic review was undertaken of publications between January 1, 1995 and December 31, 2017 across PubMed, Embase and The Cochrane Library...
February 22, 2018: Infectious Diseases and Therapy
https://www.readbyqxmd.com/read/29470103/an-approach-to-children-with-pulmonary-edema-at-high-altitude
#6
Deborah R Liptzin, Steven H Abman, Ann Giesenhagen, D Dunbar Ivy
Liptzin, Deborah R., Steven H. Abman, Ann Giesenhagen, and D. Dunbar Ivy. An approach to children with pulmonary edema at high altitude. High Alt Med Biol. 00:000-000, 2018. INTRODUCTION: Diagnosis of high-altitude illness can be more challenging in children, especially those who are preverbal. Families often travel to high elevations for family vacations, either for skiing, hiking, and/or camping. They may present to their primary care providers looking for anticipatory guidance before travel or may follow-up after developing high-altitude illness...
February 22, 2018: High Altitude Medicine & Biology
https://www.readbyqxmd.com/read/29465631/near-infrared-cerebral-oximetry-to-predict-outcome-after-pediatric-cardiac-surgery-a-prospective-observational-study
#7
Marine Flechet, Fabian Güiza, Dirk Vlasselaers, Lars Desmet, Stoffel Lamote, Heidi Delrue, Marc Beckers, Michaël P Casaer, Pieter Wouters, Greet van den Berghe, Geert Meyfroidt
OBJECTIVES: To assess whether near-infrared cerebral tissue oxygen saturation, measured with the FORESIGHT cerebral oximeter (CAS Medical Systems, Branford, CT) predicts PICU length of stay, duration of invasive mechanical ventilation, and mortality in critically ill children after pediatric cardiac surgery. DESIGN: Single-center prospective, observational study. SETTING: Twelve-bed PICU of a tertiary academic hospital. PATIENTS: Critically ill children and infants with congenital heart disease, younger than 12 years old, admitted to the PICU between October 2012 and November 2015...
February 19, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29464391/treatment-of-pediatric-pulmonary-hypertension
#8
REVIEW
Manish Aggarwal, R Mark Grady
Pulmonary hypertension (PH) is an increasingly recognized problem in children, particularly within tertiary pediatric hospitals. This increase is, in large part, due to ever improving survival among previously fatal conditions, such as extreme prematurity and complicated congenital heart disease. This increased recognition has paralleled burgeoning pharmacologic and interventional PH-specific treatment options. Unfortunately, most PH-specific therapies have not been tested in children with rigorous, randomized, controlled trials...
February 20, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29461753/-kawasaki-disease-an-update
#9
Sabrina Bressieux-Degueldre, Damien Schaffner, Michaël Hofer, Nicole Sekarski, Federica Vanoni
The cause of Kawasaki disease (KD) is still unknown, but an infectious origin and genetic susceptibility have been suggested. The American Heart Association has changed the guidelines for diagnosis and treatment of KD in 2017. One goal is to better identify patients most at risk, particularly young children with « incomplete » KD. In case of intravenous immunoglobulin resistance, alternative treatments to the traditional use of corticosteroids, such as TNF-alpha inhibitors have been proposed. For all patients with coronary aneurisms, regular assessment of myocardial perfusion is recommended, either with stress echocardiography, MRI, scintigraphy or PET-scan...
February 14, 2018: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29461227/-the-differentiated-approach-to-prevention-of-neural-tube-defects-in-children
#10
N Kotova, V Maichuk, O Fedorenko
Neural tube defects occupy second place in frequency after the defects of the cardiovascular system. The folate metabolism violation and hyperhomocysteinemia in women are proved to be the leading risk factors for the NTD of the fetus. Polymorphism of the 5,10-methylenetetrahydrofolate reductase gene (MTHFR) is a genetic determinant of folate metabolism violation. Admission of folic acid in a standard dose of 0.4 mg and / or the use of fortified foods does not allow reaching the protective level of folic acid if there is a mutation of the MTHFR gene or when several risk factors combine, which requires a higher dose of folic acid...
January 2018: Georgian Medical News
https://www.readbyqxmd.com/read/29460801/effects-of-glucocorticoids-on-serum-amino-acid-levels-during-cardiac-surgery-in-children
#11
Annelies van Zwol, Neelke B C Oosterloo, Cartijn T de Betue, Ad Bogers, Inge I de Liefde, Nicolaas E P Deutz, Koen F M Joosten
BACKGROUND AND AIMS: Children undergoing cardiac surgery with cardio pulmonary bypass often receive glucocorticoids to reduce the systemic inflammatory response. Glucocorticoids stimulate protein breakdown and increase amino acid availability. We studied whether glucocorticoid treatment influences the availability of amino acids, specifically those involved in the nitric oxide pathway. METHODS: We prospectively studied 49 children with congenital heart disease undergoing cardiac surgery...
February 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29457852/successful-management-of-living-donor-liver-transplantation-for-biliary-atresia-with-single-ventricle-physiology-from-peri-transplant-through-total-cavopulmonary-connection-a-case-report
#12
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
February 19, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29457357/improving-outpatient-advance-care-planning-for-adults-with-congenital-or-pediatric-heart-disease-followed-in-a-pediatric-heart-failure-and-transplant-clinic
#13
Lindsay A Edwards, Christine Bui, Antonio G Cabrera, Jill Ann Jarrell
OBJECTIVE: To improve outpatient advanced care planning (ACP) for adults with congenital/pediatric heart disease followed in a pediatric heart failure (HF) and transplant clinic through quality improvement (QI) methodology. DESIGN: A one-year QI project was completed. We conducted quarterly chart reviews and incorporated feedback from the providers to direct subsequent interventions. PATIENTS AND SETTING: Patients ≥18 years of age seen in the HF and Transplant Clinic for follow-up visit were included in analysis...
February 18, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29456929/controversies-in-diagnosis-and-management-of-kawasaki-disease
#14
REVIEW
Rakesh Kumar Pilania, Dharmagat Bhattarai, Surjit Singh
Kawasaki disease (KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etiology of this disorder remains a mystery. Though typical presentation of KD is quite characteristic, it may also present as incomplete or atypical disease in which case the diagnosis can be very challenging. As both incomplete and atypical forms of KD can be associated with serious coronary artery complications, the pediatrician can ill afford to miss these diagnoses...
February 8, 2018: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/29456231/late-onset-choreoathetotic-syndrome-following-heart-surgery-in-adults-with-end-stage-renal-disease
#15
Mohamed Amine Hamzi, Kawtar Hassani, Driss El Kabbaj
Choreoathetotic syndrome is a rare complication of open cardiac surgery that is seen usually in children after surgery for congenital cardiac anomalies. Here, we report two cases of adult patients with end-stage renal disease (ESRD) on regular hemodialysis who developed acute choreoathetotic syndrome few days after cardiac surgeries under cardiopulmonary bypass (CPB). Improvement was seen after an interval with complete resolution in one case. Investigations of the cause have been noncontributory. Long CPB time seems to be the main identified risk factor in these cases...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29454680/cardiomyopathy-in-the-pediatric-patients
#16
REVIEW
Shi-Min Yuan
Pediatric cardiomyopathies are a group of myocardial diseases with complex taxonomies. Cardiomyopathy can occur in children at any age, and it is a common cause of heart failure and heart transplantation in children. The incidence of pediatric cardiomyopathy is increasing with time. They may be associated with variable comorbidities, which are most often arrhythmia, heart failure, and sudden death. Medical imaging technologies, including echocardiography, cardiac magnetic resonance, and nuclear cardiology, are helpful in reaching a diagnosis of cardiomyopathy...
January 31, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29452463/autosomal-dominant-mannose-binding-lectin-deficiency-is-associated-with-worse-neurodevelopmental-outcomes-after-cardiac-surgery-in-infants
#17
Daniel Seung Kim, Yatong K Li, Jerry H Kim, Curtis S Bergquist, Marsha Gerdes, Judy C Bernbaum, Nancy Burnham, Donna M McDonald-McGinn, Elaine H Zackai, Susan C Nicolson, Thomas L Spray, Deborah A Nickerson, Hakon Hakonarson, Gail P Jarvik, J William Gaynor
OBJECTIVES: The MBL2 gene is the major genetic determinant of mannose-binding lectin (MBL)-an acute phase reactant. Low MBL levels have been associated with adverse outcomes in preterm infants. The MBL2Gly54Asp missense variant causes autosomal dominant MBL deficiency. We tested the hypothesis that MBL2Gly54Asp is associated with worse neurodevelopmental outcomes after cardiac surgery in neonates. METHODS: This is an analysis of a previously described cohort of patients with nonsyndromic congenital heart disease who underwent cardiac surgery with cardiopulmonary bypass before age 6 months (n = 295)...
March 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29446208/study-rationale-design-and-pre-transplant-alloantibody-status-a-first-report-of-clinical-trials-in-organ-transplantation-in-children-04-ctotc-04-in-pediatric-heart-transplantation
#18
Warren A Zuckerman, Adriana Zeevi, Kristen L Mason, Brian Feingold, Carol Bentlejewski, Linda J Addonizio, Elizabeth D Blume, Charles E Canter, Anne I Dipchand, Daphne T Hsu, Robert E Shaddy, William T Mahle, Anthony J Demetris, David M Briscoe, Thalachallour Mohanakumar, Joseph M Ahearn, David N Iklé, Brian D Armstrong, Yvonne Morrison, Helena Diop, Jonah Odim, Steven A Webber
Anti-HLA donor-specific antibodies are associated with worse outcomes following organ transplantation. Among sensitized pediatric heart candidates, requirement for negative donor-specific cytotoxicity crossmatch increases wait times and mortality. However, transplantation with positive crossmatch may increase post-transplant morbidity and mortality. We address this clinical challenge in a prospective, multi-center, observational cohort study of children listed for heart transplantation (CTOTC-04). Outcomes were compared among sensitized recipients who underwent transplant with positive crossmatch, non-sensitized recipients, and sensitized recipients without positive crossmatch...
February 15, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29444925/increasing-incidence-and-prevalence-of-world-health-organization-groups-1-to-4-pulmonary-hypertension-a-population-based-cohort-study-in-ontario-canada
#19
D Thiwanka Wijeratne, Katherine Lajkosz, Susan B Brogly, M Diane Lougheed, Li Jiang, Ahmad Housin, David Barber, Ana Johnson, Katharine M Doliszny, Stephen L Archer
BACKGROUND: The World Health Organization recognizes 5 groups of pulmonary hypertension (PH), categorized by pathogenesis or comorbidity: 1-pulmonary arterial hypertension 2-left-heart disease, 3-lung disease and hypoxia 4-chronic thromboembolic disease, and 5-miscellaneous. The epidemiology of PH, apart from group 1, is largely unknown. METHODS AND RESULTS: We describe incidence, prevalence, comorbidities, mortality and prescribing patterns for groups 1 to 4 PH from 1993 to 2012...
February 2018: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/29444807/hypoxic-challenge-testing-fitness-to-fly-in-children-with-complex-congenital-heart-disease
#20
Nitha N Naqvi, Victoria L Doughty, Luke Starling, Rodney C Franklin, Simon Ward, Piers E F Daubeney, Ian M Balfour-Lynn
OBJECTIVE: Commercial airplanes fly with an equivalent cabin fraction of inspired oxygen of 0.15, leading to reduced oxygen saturation (SpO2 ) in passengers. How this affects children with complex congenital heart disease (CHD) is unknown. We conducted Hypoxic Challenge Testing (HCT) to assess need for inflight supplemental oxygen. METHODS: Children aged <16 years had a standard HCT. They were grouped as (A) normal versus abnormal baseline SpO2 (≥95% vs <95%) and (B) absence versus presence of an actual/potential right-to-left (R-L) shunt...
February 14, 2018: Heart: Official Journal of the British Cardiac Society
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