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Pediatric cardiomyopathy

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https://www.readbyqxmd.com/read/29032583/third-generation-ventricular-assist-device-mid-term-outcomes-of-the-heartware-hvad-in-pediatric-patients
#1
Mustafa Pac, Sinan Sabit Kocabeyoglu, Umit Kervan, Dogan Emre Sert, Serhat Koca, Ibrahim Ece, Feyza Aysenur Pac
The HeartWare HVAD is a small, third generation continuous flow pump that is intracorporeally placed for support of a failing ventricle in adult patients. This device is small in size when compared to other left ventricular assist devices and can therefore be used in smaller sized pediatric patients. We present our initial experience using the HVAD as a bridge to heart transplantation in the pediatric population. We performed a retrospective, single center, nonrandomized review of 17 pediatric patients who underwent HVAD implantation between June 2013 and March 2016...
October 15, 2017: Artificial Organs
https://www.readbyqxmd.com/read/29019615/haemodynamic-profiles-of-children-with-end-stage-heart-failure
#2
Sharon Chen, John C Dykes, Doff B McElhinney, Robert J Gajarski, Andrew Y Shin, Seth A Hollander, Melanie E Everitt, Jack F Price, Ravi R Thiagarajan, Steven J Kindel, Joseph W Rossano, Beth D Kaufman, Lindsay J May, Elizabeth Pruitt, David N Rosenthal, Christopher S Almond
Aims: To evaluate associations between haemodynamic profiles and symptoms, end-organ function and outcome in children listed for heart transplantation. Methods and results: Children <18 years listed for heart transplant between 1993 and 2013 with cardiac catheterization data [pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and cardiac index (CI)] in the Pediatric Heart Transplant Study database were included. Outcomes were New York Heart Association (NYHA)/Ross classification, renal and hepatic dysfunction, and death or clinical deterioration while on waitlist...
October 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28986271/the-subclinical-cardiomyopathy-of-friedreich-s-ataxia-in-a-pediatric-population
#3
Jonathan F Plehn, Keren Hasbani, Inez Ernst, Kenneth D Horton, Bart E Drinkard, Nicholas A Di Prospero
BACKGROUND: Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described. METHODS: We performed echocardiography (echo), cardiac magnetic resonance imaging (cMRI) and neurologic assessment in a cross-sectional analysis of 48 genetically-confirmed FA subjects aged 9-17 years with moderate neurologic impairment but without a cardiovascular history. Echo and cMRI-determined left ventricular mass were indexed to height in g/m2...
October 3, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28971158/scorpion-related-cardiomyopathy-and-acute-pulmonary-edema-in-a-child-who-is-stung-by-leiurus-abdullahbayrami
#4
Mehmet Dokur, Mustafa Dogan, Ersen Aydin Yagmur
Venom of Leiurus abdullahbayrami (Scorpiones: Buthidae) is an extremely toxic one and it stimulates voltage-gated sodium and potassium channels. In case of a stung by this scorpion; excessive catecholamine release occur and it impairs left ventricle contractility and consequently a heart failure occurs (scorpion sting-related cardiomyopathy). In addition to this cardiac-induced acute pulmonary, edema may occur in severe cases too. An 11-year-old male child who was stung by a scorpion (species: Leiurus abdullahbayrami) consulted to the Emergency Room...
September 2017: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28965736/second-annual-pediatric-interagency-registry-for-mechanical-circulatory-support-pedimacs-report-pre-implant-characteristics-and-outcomes
#5
Elizabeth D Blume, Christina VanderPluym, Angela Lorts, J Timothy Baldwin, Joseph W Rossano, David L S Morales, Ryan S Cantor, Marissa A Miller, James D St Louis, Devin Koehl, David L Sutcliffe, Pirooz Eghtesady, James K Kirklin, David N Rosenthal
BACKGROUND: Expanded use of pediatric ventricular assist devices (VADs) has decreased mortality in children awaiting heart transplantation. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Heart, Lung, and Blood Institute-sponsored North American database, provides a platform to understand this emerging population. METHODS: Between September 2012 and September 2016, patients aged younger than 19 years who underwent VAD implantation were enrolled in Pedimacs...
July 4, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28964475/pediatric-cardiac-transplantation
#6
Thomas D Ryan, Clifford Chin
Heart transplantation in pediatric patients generally arises as a treatment option of last resort, that is, the indication is for patients with heart failure of various etiologies, with potential or actual end-organ dysfunction, in whom there are no reasonable, long-term options for life-prolonging therapy. The concept of heart failure is complex in a pediatric population, particularly those with congenital heart disease. While heart failure may refer simply to systolic dysfunction leading to low cardiac output, it can also encompass: diastolic dysfunction in restrictive cardiomyopathy; single ventricle physiology without an option for stable palliation...
August 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28958965/bnp-response-during-berlin-heart-%C3%A2-support-in-child-a-case-report
#7
Nicolas Beranger, Charlotte Veyrat-Durebex, Philippe Pouard, Bernard Lacour, Stéphanie Vicca
A five-year-old boy is presented to Necker hospital for a dilated hypertrophic cardiomyopathy. The implantation of the Berlin Heart Excor(®) ventricular assist device was performed. This pediatric-sized Berlin Heart(®) device provides mechanical support for young infants and children of all ages to sustain the failing cardiac circulation over several months, until either recovery of myocardial function or heart transplantation. It remains difficult to identify patients with sufficient recovery and the right time for explantation of the Berlin Heart(®)...
October 1, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/28942941/iron-laboratory-studies-in-pediatric-patients-with-heart-failure-from-dilated-cardiomyopathy
#8
David Higgins, Jessica Otero, Christa Jefferis Kirk, Jennifer Pak, Neal Jorgensen, Mariska Kemna, Erin Albers, Borah Hong, Joshua Friedland-Little, Yuk Law
Iron deficiency (FeD), with or without anemia, in adults with heart failure (HF) is associated with poor outcomes, which can be improved with replacement therapy. A similar therapeutic opportunity may exist for children; however, iron laboratory measurements and FeD have not been described in pediatric patients with HF. A single-center, retrospective study was conducted on 28 patients <21 years old with a diagnosis of dilated cardiomyopathy and HF who had iron laboratories (serum iron, iron saturation, and ferritin) performed...
August 30, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28939701/rapid-targeted-genomics-in-critically-ill-newborns
#9
Cleo C van Diemen, Wilhelmina S Kerstjens-Frederikse, Klasien A Bergman, Tom J de Koning, Birgit Sikkema-Raddatz, Joeri K van der Velde, Kristin M Abbott, Johanna C Herkert, Katharina Löhner, Patrick Rump, Martine T Meems-Veldhuis, Pieter B T Neerincx, Jan D H Jongbloed, Conny M van Ravenswaaij-Arts, Morris A Swertz, Richard J Sinke, Irene M van Langen, Cisca Wijmenga
BACKGROUND: Rapid diagnostic whole-genome sequencing has been explored in critically ill newborns, hoping to improve their clinical care and replace time-consuming and/or invasive diagnostic testing. A previous retrospective study in a research setting showed promising results with diagnoses in 57%, but patients were highly selected for known and likely Mendelian disorders. The aim of our prospective study was to assess the speed and yield of rapid targeted genomic diagnostics for clinical application...
October 2017: Pediatrics
https://www.readbyqxmd.com/read/28928612/human-herpesvirus-6-induced-inflammatory-cardiomyopathy-in-immunocompetent-children
#10
REVIEW
Surabhi Reddy, Eva Eliassen, Gerhard R Krueger, Bibhuti B Das
Over the last decade, human herpesvirus 6 (HHV-6) has been implicated in the etiology of pediatric myocarditis and subsequent dilated cardiomyopathy (DCM). This review provides an overview of recent literature investigating the pathophysiological relevance of HHV-6 in inflammatory cardiomyopathy. We examined 11 cases of previously published pediatric myocarditis and/or DCM associated with HHV-6 and also our experience of detection of virus particles in vascular endothelium of HHV-6 positive endomyocardial biopsy tissue by electron microscopy...
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#11
REVIEW
Teresa M Lee, Daphne T Hsu, Paul Kantor, Jeffrey A Towbin, Stephanie M Ware, Steven D Colan, Wendy K Chung, John L Jefferies, Joseph W Rossano, Chesney D Castleberry, Linda J Addonizio, Ashwin K Lal, Jacqueline M Lamour, Erin M Miller, Philip T Thrush, Jason D Czachor, Hiedy Razoky, Ashley Hill, Steven E Lipshultz
Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28905470/outcomes-of-berlin-heart-excor-%C3%A2-pediatric-ventricular-assist-device-support-in-patients-with-restrictive-and-hypertrophic-cardiomyopathy
#12
Jennifer A Su, Jondavid Menteer
The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR(®) ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR(®) were identified...
September 14, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28874164/radiation-free-cmr-diagnostic-heart-catheterization-in-children
#13
Kanishka Ratnayaka, Joshua P Kanter, Anthony Z Faranesh, Elena K Grant, Laura J Olivieri, Russell R Cross, Ileen F Cronin, Karin S Hamann, Adrienne E Campbell-Washburn, Kendall J O'Brien, Toby Rogers, Michael S Hansen, Robert J Lederman
BACKGROUND: Children with heart disease may require repeated X-Ray cardiac catheterization procedures, are more radiosensitive, and more likely to survive to experience oncologic risks of medical radiation. Cardiovascular magnetic resonance (CMR) is radiation-free and offers information about structure, function, and perfusion but not hemodynamics. We intend to perform complete radiation-free diagnostic right heart catheterization entirely using CMR fluoroscopy guidance in an unselected cohort of pediatric patients; we report the feasibility and safety...
September 6, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28859790/ivabradine-in-children-with-dilated-cardiomyopathy-and-symptomatic-chronic-heart-failure
#14
RANDOMIZED CONTROLLED TRIAL
Damien Bonnet, Felix Berger, Eero Jokinen, Paul F Kantor, Piers E F Daubeney
BACKGROUND: Heart rate reduction as a therapeutic target has been investigated in adults with heart failure (HF). Ivabradine has shown promising efficacy, but has not been evaluated in children. Currently, treatment recommendations for chronic pediatric HF are based mainly on chronic HF guidelines for adults. OBJECTIVES: The authors explored the dose-response relationship of ivabradine in children with dilated cardiomyopathy and symptomatic chronic HF. The primary endpoint was ≥20% reduction in heart rate from baseline without inducing bradycardia or symptoms...
September 5, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28840327/the-safety-and-effectiveness-of-flecainide-in-children-in-the-current-era
#15
Taylor Cunningham, Orhan Uzun, Rachel Morris, Sonia Franciosi, Amos Wong, Ida Jeremiasen, Elizabeth Sherwin, Shubhayan Sanatani
This retrospective study sought to determine the safety and effectiveness of flecainide in children with normal hearts and those with congenital heart disease (CHD) or cardiomyopathy (CMO). Baseline and follow-up data at two pediatric cardiology sites were queried (2000-2015); a total of 175 patients (20 with CHD and two with CMO) receiving flecainide were assessed. When comparing patients with CHD to those with normal hearts, patients with CHD were younger at diagnosis (median age 19 days; IQR 3-157.5 days vs normal heart patients median age 21 days; IQR 7-172 days, p = 0...
August 24, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28802483/left-ventricular-systolic-myocardial-deformation-a%C3%A2-comparison-of-two-and-three-dimensional-echocardiography-in-children
#16
Sowmya Balasubramanian, Rajesh Punn, Shea N Smith, Helene Houle, Theresa A Tacy
BACKGROUND: The clinical applicability and reliability of three-dimensional (3D) speckle-tracking echocardiography has not been well studied in pediatric patients. The aim of this study was to compare two-dimensional (2D) echocardiography and 3DE real-time full-volume-derived strain and rotation indices in healthy children and patients with dilated cardiomyopathy (DCM). METHODS: Children with either normal function or DCM were prospectively recruited in an outpatient setting, and deformation indices, including circumferential, radial, and longitudinal strain and torsion, were measured by 2D and 3D echocardiography...
October 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28764973/disturbed-desmoglein-2-in-the-intercalated-disc-of-pediatric-patients-with-dilated-cardiomyopathy
#17
Elise L Kessler, Peter Gj Nikkels, Toon Ab van Veen
Dilated cardiomyopathy (DCM) leads to disturbed contraction and force transduction, and is associated with substantial mortality in all age groups. Involvement of a disrupted composition of the intercalated disc (ID) has been reported. However, in children, little is established about such subcellular changes during disease, because of the pathological mix-up with the ongoing cardiac maturation. This leaves maladaptive remodeling often undetected. We aimed at illustrating subcellular alterations in children diagnosed with DCM compared to age-matched controls, focusing on ID proteins known to be crucially stable under healthy conditions and destabilized during cardiac injury in adults...
September 2017: Human Pathology
https://www.readbyqxmd.com/read/28762167/elevated-myocardial-extracellular-volume-fraction-in-duchenne-muscular-dystrophy
#18
James J Starc, Ryan A Moore, Mantosh S Rattan, Chet R Villa, Zhiqian Gao, Wojciech Mazur, John L Jefferies, Michael D Taylor
Duchenne muscular dystrophy (DMD) is a genetic, X-linked recessive disease with an associated cardiomyopathy characterized by myocardial fibrosis leading to heart failure, arrhythmias, and death. Earlier detection and treatment of cardiac involvement in DMD hold potential to improve outcomes. Cardiovascular magnetic resonance (CMR) extracellular volume (ECV) quantification using T1 mapping is a histologically validated, non-invasive marker of diffuse fibrosis. This study aims to determine the ECV in a pediatric DMD population, and correlate it with metrics of left ventricular function...
July 31, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28755194/hereditary-tyrosinemia-type-1-in-turkey
#19
Ayse Cigdem Aktuglu-Zeybek, Ertugrul Kiykim, M Serif Cansever
Hereditary tyrosinemia type 1 (HT1, OMIM 276700) is a rare autosomal recessively inherited inborn error of metabolism in the tyrosine catabolic pathway due to deficiency of the enzyme fumarylacetoacetate hydrolase. The clinical features of HT1 are widely heterogenous even within the same family members. Clinical features includes acute or chronic liver disease with increased risk of hepatocellular carcinoma, hypophosphatemic rickets due to renal tubular dysfunction, glomerulosclerosis, failure to thrive, neurological porphyria-like crisis, hypertrophic cardiomyopathy and hypoglycemia due to hyperinsulinism...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28737196/-results-of-a-national-program-of-pediatric-heart-transplantation-strengths-and-weakness
#20
Pedro Becker, Santiago Besa, Sergio Riveros, Rodrigo González, Alfonso Navia, Paulina Dellepiane, Daniel Springmuller, Gonzalo Urcelay
Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. OBJECTIVES: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. PATIENTS AND METHODS: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality...
June 2017: Revista Chilena de Pediatría
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