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Pediatric cardiomyopathy

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https://www.readbyqxmd.com/read/29668027/heart-transplant-in-children-what-a-primary-care-provider-needs-to-know
#1
Swati Sehgal, Emily Shea, Lauren Kelm, Deepak Kamat
Heart transplantation is offered to children with heart failure that is not amenable to medical or surgical therapy. Indications for heart transplant include unrepairable congenital heart disease, failed palliation of congenital heart disease, and cardiomyopathies. There has been tremendous progress in this field since the first heart transplant was performed in 1967. Each year, approximately 500 pediatric heart transplants take place worldwide. Pediatric heart transplant survivors are living longer with their initial transplant...
April 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29651540/identifying-non-invasive-tools-to-distinguish-acute-myocarditis-from-dilated-cardiomyopathy-in-children
#2
Divya Suthar, Debra A Dodd, Justin Godown
There is often a diagnostic dilemma in pediatric patients presenting with depressed ventricular function, as myocarditis and dilated cardiomyopathy (DCM) of other etiologies can appear very similar. Accurate identification is critical to guide treatment and to provide families with the most accurate expectation of long-term outcomes. The objective of this study was to identify patterns of clinical presentation and to assess non-invasive measures to differentiate patients with acute myocarditis from other forms of DCM...
April 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29622585/prevalence-and-progression-of-late-gadolinium-enhancement-in-children-and-adolescents-with-hypertrophic-cardiomyopathy
#3
Anna Axelsson Raja, Hoshang Farhad, Anne Marie Valente, John-Paul Couce, John Lynn Jefferies, Henning Bundgaard, Kenneth Zahka, Harry Lever, Anne M Murphy, Euan Ashley, Sharlene M Day, Mark V Sherrid, Ling Shi, David A Bluemke, Charles E Canter, Steven D Colan, Carolyn Y Ho
Background -Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ~60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. Additionally, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM...
April 5, 2018: Circulation
https://www.readbyqxmd.com/read/29607544/outcome-of-pediatric-heart-transplantation-in-blood-culture-positive-donors-in-the-united-states
#4
Shahnawaz M Amdani, Wei Du, Sanjeev Aggarwal
Active donor infection at time of organ procurement poses a potential infection risk and may increase post-transplant morbidity and mortality in recipients. Our hypothesis was that pediatric heart transplant recipients from blood culture positive donors (BCPD) would have increased morbidity and mortality compared to non-blood culture positive donors (NBCPD). A retrospective analyses of pediatric heart transplant recipients using the Organ Procurement and Transplant Network (OPTN) between 1987 and 2015 were conducted...
April 1, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29606334/apical-transverse-motion-is-associated-with-interventricular-mechanical-delay-and-decreased-left-ventricular-function-in-children-with-dilated-cardiomyopathy
#5
Wei Hui, Cameron Slorach, Mark K Friedberg
BACKGROUND: Apical transverse motion (ATM) is associated with electromechanical dyssynchrony in adult dilated cardiomyopathy (DCM). Bundle branch block electromechanical dyssynchrony is uncommon in pediatric DCM, but ATM and its association with ventricular function have not been characterized. METHODS: Fifty-six children with DCM were retrospectively studied. Using echocardiography, ATM was assessed visually and by speckle-tracking longitudinal displacement of the interventricular septal and left ventricular (LV) lateral walls in opposite directions...
March 29, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29602613/noninvasive-echocardiographic-measures-of-pulmonary-vascular-resistance-in-children-and-young-adults-with-cardiomyopathy
#6
Dor Markush, Robert D Ross, Ronald Thomas, Sanjeev Aggarwal
BACKGROUND: Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM. METHODS: Imaging studies were retrospectively reviewed from pediatric patients with CM who underwent both echocardiography and cardiac catheterization within a 72-hour period...
March 27, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29587521/orthotopic-heart-transplantation-in-children
#7
Nagarajan Muthialu
Heart transplantation is a relatively infrequent but technically demanding procedure in pediatric cardiac surgery. Orthotopic heart transplantation is often offered to older children or adolescents following multiple previous operations, but with advances in intensive care, diagnostics, and management, more infants with complex congenital heart diseases are being listed for transplantation. Primary cardiomyopathies remain the most common indication. Outcomes following cardiothoracic transplantation have improved steadily in recent times...
January 1, 2018: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/29581480/late-cardiovascular-morbidity-and-mortality-following-pediatric-allogeneic-hematopoietic-cell-transplantation
#8
Christine N Duncan, Ruta Brazauskas, Jiaxing Huang, Bronwen E Shaw, Navneet S Majhail, Bipin N Savani, Mary E D Flowers, Minoo Battiwalla, Kristen Beebe, Andrew C Dietz, Christopher C Dvorak, Roger Giller, David A Jacobsohn, Morris Kletzel, Paul L Martin, Eneida R Nemecek, Brandon Nuechterlein, Julie-An Talano, Michael A Pulsipher, K Scott Baker
We analyzed late cardiovascular outcomes of 661 patients who survived at least 2 years from hematopoietic cell transplantation for childhood hematologic malignancy between 1995 and 2008. Center for International Blood and Marrow Transplant Research data was supplemented with surveys focused on cardiotoxicity and potential risk factors. The median duration of follow-up was 97 months (range 24-230). 4.2% of survivors experienced at least one of the primary outcomes including coronary artery disease (0.2%), cerebrovascular accident (0...
March 26, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29567486/genetic-evaluation-of-cardiomyopathy-a-heart-failure-society-of-america-practice-guideline
#9
Ray E Hershberger, Michael Givertz, Carolyn Y Ho, Daniel P Judge, Paul Kantor, Kim L McBride, Ana Morales, Matthew R G Taylor, Matteo Vatta, Stephanie M Ware
This guideline describes the approach and expertise needed for the genetic evaluation of cardiomyopathy. First published in 2009 by the Heart Failure Society of America (HFSA), this guidance has now been updated in collaboration with the American College of Medical Genetics and Genomics (ACMG). The writing group, composed of cardiologists and genetics professionals with expertise in adult and pediatric cardiomyopathy, reflects the emergence and increased clinical activity devoted to cardiovascular genetic medicine...
March 19, 2018: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/29564593/advances-in-heart-failure-therapy-in-pediatric-patients-with-dilated-cardiomyopathy
#10
REVIEW
Stefan Rupp, Christian Jux
While in adults major advances in heart failure therapy in patients with dilated cardiomyopathy were documented in the last two decades, research on the mechanism and therapies of heart failure in children with left ventricular dilated cardiomyopathy has lagged behind. Despite the lack of sufficient randomized prospective studies, ACE inhibitors are first line and ß-receptor antagonists are second-line strategies in children. Following the adult guidelines, without having data concerning the pediatric population, mineral corticoids are also accepted in the treatment of pediatric heart failure, while diuretics should only be used to achieve a euvolemic status...
March 22, 2018: Heart Failure Reviews
https://www.readbyqxmd.com/read/29552945/predictors-of-poor-outcomes-in-pediatric-venoarterial-extracorporeal-membrane-oxygenation
#11
Maanasi S Mistry, Sara M Trucco, Timothy Maul, Mahesh S Sharma, Li Wang, Shawn West
BACKGROUND: Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides respiratory and hemodynamic support to pediatric patients in severe cardiac failure. We aim to identify risk factors associated with poorer outcomes in this population. METHODS: A retrospective chart review was conducted of pediatric patients requiring VA-ECMO support for cardiac indications at our institution from 2004 to 2015. Data were collected on demographics, indication, markers of cardiac output, ventricular assist device (VAD) insertion, heart transplantation, or left atrial (LA) decompression...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29551744/world-wide-experience-of-a-durable-centrifugal-flow-pump-in-pediatric-patients
#12
J Conway, O Miera, I Adachi, K Maeda, P Eghtesady, H T Henderson, K Guleserian, C-P S Fan, R Kirk
OBJECTIVES: The primary objectives of this study were to describe the characteristics and survival outcomes for children supported with the HeartWare HVAD® system from the global community. METHODS: This was a retrospective survey of patients <18 years with a HVAD® system. Questionnaires were sent to sites across the world in April 2015 and collected between May 2015-16. Information on 205 patients was collected. RESULTS: The median age of implant was 13...
March 15, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29547457/retrospective-application-of-new-pediatric-ventilator-associated-pneumonia-criteria-identifies-a-high-risk-population
#13
Ashley Gionfriddo, Mika L Nonoyama, Peter C Laussen, Peter N Cox, Megan Clarke, Alejandro A Floh
OBJECTIVES: To promote standardization, the Centers for Disease Control and Prevention introduced a new ventilator-associated pneumonia classification, which was modified for pediatrics (pediatric ventilator-associated pneumonia according to proposed criteria [PVAP]). We evaluated the frequency of PVAP in a cohort of children diagnosed with ventilator-associated pneumonia according to traditional criteria and compared their strength of association with clinically relevant outcomes. DESIGN: Retrospective cohort study...
March 15, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29536133/zinc-and-zinc-transporters-novel-regulators-of-ventricular-myocardial-development
#14
Wen Lin, Deqiang Li
Ventricular myocardial development is a well-orchestrated process involving different cardiac structures, multiple signal pathways, and myriad proteins. Dysregulation of this important developmental event can result in cardiomyopathies, such as left ventricle non-compaction, which affect the pediatric population and the adults. Human and mouse studies have shed light upon the etiology of some cardiomyopathy cases and highlighted the contribution of both genetic and environmental factors. However, the regulation of ventricular myocardial development remains incompletely understood...
March 13, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29517769/toward-an-effective-exome-based-genetic-testing-strategy-in-pediatric-dilated-cardiomyopathy
#15
Johanna C Herkert, Kristin M Abbott, Erwin Birnie, Martine T Meems-Veldhuis, Ludolf G Boven, Marloes Benjamins, Gideon J du Marchie Sarvaas, Daniela Q C M Barge-Schaapveld, J Peter van Tintelen, Paul A van der Zwaag, Yvonne J Vos, Richard J Sinke, Maarten P van den Berg, Irene M van Langen, Jan D H Jongbloed
PurposeWe evaluated the diagnostic yield in pediatric dilated cardiomyopathy (DCM) of combining exome sequencing (ES)-based targeted analysis and genome-wide copy-number variation (CNV) analysis. Based on our findings, we retrospectively designed an effective approach for genetic testing in pediatric DCM.MethodsWe identified 95 patients (in 85 families) with pediatric onset of DCM. We initially excluded 13 of these families because they already had a genetic diagnosis, leaving a total of 31 probands for single-nucleotide polymorphism (SNP) array and trio-ES...
March 8, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29504323/electrocardiographic-manifestations-in-paediatric-wilson-disease
#16
Syeda Sara Batool Hamdani, Huma Arshad Cheema, Anjum Saeed, Hassan Suleman Malik, Tayaba Sehar
BACKGROUND: Wilson disease (WD) is one of the most common metabolic liver diseases in older children. It has a strong genetic background with autosomal recessive inheritance. WD is a multisystem disorder with predominant hepatic and neurological manifestations and variable age of presentation. The data on cardiac manifestations in children is very limited and only few adult studies are available in the literature. This study was planned to determine the frequency and spectrum of Electrocardiographic (ECG) changes in pediatric WD...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/29490994/long-term-outcomes-of-hypertrophic-cardiomyopathy-diagnosed-during-childhood-results-from-a-national-population-based-study
#17
Peta M A Alexander, Alan W Nugent, Piers E F Daubeney, Katherine J Lee, Lynn A Sleeper, Tibor Schuster, Christian Turner, Andrew M Davis, Chris Semsarian, Steven D Colan, Terry Robertson, James Ramsay, Robert Justo, Gary F Sholler, Ingrid King, Robert G Weintraub
Background -Late survival and symptomatic status of children with hypertrophic cardiomyopathy (HCM) have not been well defined. We examined long-term outcomes for pediatric HCM. Methods -The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end-point was time to death or cardiac transplantation. Results -There were 80 patients with HCM with median age at diagnosis of 0...
February 28, 2018: Circulation
https://www.readbyqxmd.com/read/29468383/effect-of-levocarnitine-on-the-therapeutic-efficacy-of-conventional-therapy-in-children-with-dilated-cardiomyopathy-results-of-a-randomized-trial-in-29-children
#18
Yuwen Wang, Yi Xu, Runmei Zou, Lijia Wu, Ping Liu, Hong Yang, Zhenwu Xie, Cheng Wang
BACKGROUND: The effect of levocarnitine supplementation has not been evaluated in children with dilated cardiomyopathy (DCM). OBJECTIVE: The aim of this study was to explore the effect of oral levocarnitine supplementation in pediatric patients with DCM. METHODS: Twenty-nine children with DCM (17 male, 12 female, aged 1 month to 13 years) were divided into two groups according to a simple randomization: control group (n = 10) and experimental group (n = 19)...
February 21, 2018: Paediatric Drugs
https://www.readbyqxmd.com/read/29454680/cardiomyopathy-in-the-pediatric-patients
#19
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/29440775/genetic-defects-in-mtdna-encoded-protein-translation-cause-pediatric-mitochondrial-cardiomyopathy-with-early-onset-brain-disease
#20
Rick Kamps, Radek Szklarczyk, Tom E Theunissen, Debby M E I Hellebrekers, Suzanne C E H Sallevelt, Iris B Boesten, Bart de Koning, Bianca J van den Bosch, Gajja S Salomons, Marisa Simas-Mendes, Rob Verdijk, Kees Schoonderwoerd, Irenaeus F M de Coo, Jo M Vanoevelen, Hubert J M Smeets
This study aims to identify gene defects in pediatric cardiomyopathy and early-onset brain disease with oxidative phosphorylation (OXPHOS) deficiencies. We applied whole-exome sequencing in three patients with pediatric cardiomyopathy and early-onset brain disease with OXPHOS deficiencies. The brain pathology was studied by MRI analysis. In consanguineous patient 1, we identified a homozygous intronic variant (c.850-3A > G) in the QRSL1 gene, which was predicted to cause abnormal splicing. The variant segregated with the disease and affected the protein function, which was confirmed by complementation studies, restoring OXPHOS function only with wild-type QRSL1...
February 13, 2018: European Journal of Human Genetics: EJHG
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