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Hypertrophic cardiomyopathy in children

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https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#1
REVIEW
Imre J Pavo, Ina Michel-Behnke
Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients...
February 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28270260/what-is-the-diagnostic-value-of-the-paediatric-exercise-tolerance-test-results-from-a-uk-centre
#2
Magdalena A Sajnach-Menke, Sarah C Walpole
Purpose The aim of this study was to determine whether the exercise tolerance test can provide diagnostic and prognostic information regarding children and young adults and help predict outcome. METHODS: A total of 87 patients, aged 7-29 years (median 13, mean 13.4) were selected retrospectively. They underwent exercise test at the Freeman Hospital from December, 2015 to May, 2016. There were two groups of patients - 46 had symptoms such as chest pain, palpitations, syncope, or dyspnoea on exertion and no cardiac diagnosis, and 40 patients had a cardiac diagnosis such as hypertrophic cardiomyopathy, transposition of the great arteries with post-arterial switch operation, aortic stenosis or regurgitation, tetralogy of Fallot, abnormal coronary arteries, Wolff-Parkinson-White syndrome, or supraventricular tachycardia...
March 8, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28261285/comparison-of-echocardiography-with-tissue-doppler-imaging-and-magnetic-resonance-imaging-with-delayed-enhancement-in-the-assessment-of-children-with-hypertrophic-cardiomyopathy
#3
Lidia Ziółkowska, Joanna Petryka, Agnieszka Boruc, Wanda Kawalec
INTRODUCTION: In children with hypertrophic cardiomyopathy (HCM) there often occurs a non-ischemic pattern of myocardial fibrosis, which could be the cause of impaired left ventricular (LV) diastolic function assessed by tissue Doppler imaging (TDI). The aim of the study was to determine the prevalence of myocardial fibrosis in children with HCM, and to evaluate its relationship with echocardiographic parameters including LV diastolic dysfunction. MATERIAL AND METHODS: Sixty-three children with HCM, mean age 12...
March 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28258649/the-cardiac-manifestations-of-inherited-metabolic-diseases-in-children
#4
REVIEW
David F A Lloyd, Roshni Vara, Sujeev Mathur
Inborn errors of metabolism (IEMs) are responsible for around 5% of all cases of cardiomyopathy (CM) and 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic cardiomyopathy, whereas the accumulation of toxic metabolites, as seen in the organic acidurias, is associated with dilated cardiomyopathy (DCM). Mixed pathology is also possible, particularly in late presentations. IEMs such as Barth syndrome, a disorder of cardiolipin stability usually associated with DCM, have been associated with rarer types of CM such as endocardial fibroelastosis (EFE) and left ventricular non-compaction...
March 4, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28146440/sudden-collapse-of-a-preschool-aged-child-on-the-playground
#5
RoseAnn L Scheller, Laurie H Johnson, Michelle C Caruso, Angela Lorts
PURPOSE: Primary cardiac events are rare in children. There is little information in the literature regarding the most appropriate emergency department (ED) management of this type of pediatric patient, especially with regard to medication use. SUMMARY: This case highlights the pediatric ED evaluation and treatment of sudden collapse in a child with an ultimate diagnosis of hypertrophic cardiomyopathy. Cardiac disorders represent 2% to 6% of cases of pediatric syncope presenting to EDs, particularly if there are previous prodromal symptoms and/or a history of exertion...
February 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28102598/novel-findings-of-left-ventricular-non-compaction-cardiomyopathy-microform-cleft-lip-and-poor-vision-in-patient-with-smc1a-associated-cornelia-de-lange-syndrome
#6
Tara L Wenger, Penny Chow, Stephanie C Randle, Anna Rosen, Craig Birgfeld, Joanna Wrede, Patrick Javid, Darcy King, Vivian Manh, Anne V Hing, Erin Albers
Relatively few patients with Cornelia de Lange syndrome (CdLS) due to SMC1A mutation have been reported, limiting understanding of the full extent of the phenotype. Compared to children with classic NIPBL-associated CdLS, patients with SMC1A-associated CdLS have a milder physical phenotype with prominent intellectual disability, high rate of cleft palate and absence of limb reductions. We present a patient with SMC1A-associated CdLS who had typical features including developmental delay, seizure disorder, feeding difficulties, hirsutism, and cleft palate...
February 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28070248/childhood-cardiomyopathies-a-study-in-tertiary-care-hospital-in-upper-egypt
#7
Mohamed Abd Elaal Bakeet, Montaser Mohamed Mohamed, Ahmed Ahmed Allam, Rania Gamal
INTRODUCTION: Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital...
November 2016: Electronic Physician
https://www.readbyqxmd.com/read/27974896/echocardiography-as-a-screening-test-for-myocardial-scarring-in-children-with-hypertrophic-cardiomyopathy
#8
Gregory Compton, Lynne Nield, Andreea Dragulescu, Lee Benson, Lars Grosse-Wortmann
Introduction. Hypertrophic cardiomyopathy (HCM) is burdened with morbidity and mortality including tachyarrhythmias and sudden cardiac death. These complications are attributed in part to the formation of proarrhythmic scars in the myocardium. The presence of extensive LGE is a risk factor for adverse outcomes in HCM. Late gadolinium enhancement (LGE) cardiac magnetic resonance imaging (cMRI) is the standard for the noninvasive evaluation of myocardial scars. However, echocardiography represents an attractive screening tool for myocardial scarring...
2016: International Journal of Pediatrics
https://www.readbyqxmd.com/read/27789109/myocardial-dimensions-in-children-with-hypertrophic-cardiomyopathy-a-comparison-between-echocardiography-and-cardiac-magnetic-resonance-imaging
#9
Jonathan D Windram, Andreea Dragelescu, Lee Benson, Jonathan Forsey, Masoud Shariat, Shi-Joon Yoo, Luc Mertens, Derek Wong, Lars Grosse-Wortmann
BACKGROUND: The primary mode of imaging in hypertrophic cardiomyopathy (HCM) is transthoracic echocardiography (TTE). However, in adults inadequate acoustic windows lead to poor quantification of myocardial thickness compared with cardiac magnetic resonance (CMR) imaging. In comparison, children have better acoustic windows and TTE measurements of wall thickness might be more accurate. The aim of this study was to assess the performance of TTE compared with CMR for the assessment of myocardial thickness in children with HCM...
December 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27769300/a-homozygous-splicing-mutation-in-elac2-suggests-phenotypic-variability-including-intellectual-disability-with-minimal-cardiac-involvement
#10
Nadia A Akawi, Salma Ben-Salem, Jozef Hertecant, Anne John, Thachillath Pramathan, Praseetha Kizhakkedath, Bassam R Ali, Lihadh Al-Gazali
BACKGROUND: The group of ELAC2-related encephalomyopathies is a recent addition to the rapidly growing heterogeneous mitochondrial disorders. RESULTS: We describe a highly inbred consanguineous Pakistani family with multiple affected children in 2 branches exhibiting moderately severe global developmental delay. Using homozygosity mapping, we mapped the phenotype in this family to a single locus on chromosome 17. In addition, whole-exome sequencing identified a homozygous splicing mutation (c...
October 21, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27721865/retrospective-multicenter-study-of-respiratory-syncytial-virus-prophylaxis-in-korean-children-with-congenital-heart-diseases
#11
Ah Young Kim, Se Yong Jung, Jae Young Choi, Gi Beom Kim, Young-Hwue Kim, Woo Sup Shim, I-Seok Kang, Jo Won Jung
BACKGROUND AND OBJECTIVES: We conducted a review of current data on respiratory syncytial virus (RSV) prophylaxis with palivizumab, in Korean children with congenital heart diseases (CHD). In 2009, the Korean guideline for RSV prophylaxis had established up to five shots monthly per RSV season, only for children <1 year of age with hemodynamic significance CHD (HS-CHD). SUBJECTS AND METHODS: During the RSV seasons in 2009-2015, we performed a retrospective review of data for 466 infants with CHD, examined at six centers in Korea...
September 2016: Korean Circulation Journal
https://www.readbyqxmd.com/read/27659477/t1-mapping-in-children-and-young-adults-with-hypertrophic-cardiomyopathy
#12
Keyur Parekh, Michael Markl, Jie Deng, Roger A de Freitas, Cynthia K Rigsby
To assess the global and segmental left ventricular (LV) native T1 and extracellular volume fraction (ECV) in children and young adults with hypertrophic cardiomyopathy (HCM) compared to a control cohort. The study population included 21 HCM patients (mean 14.1 ± 4.6 years) and 21 controls (mean 15.7 ± 1.5 years). Native modified Look-Locker inversion recovery sequence was performed before and after contrast injection in 3 short axis planes. Global and segmental LV native T1 and ECV were quantified and compared between HCM patients and controls...
January 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/27645989/a-novel-method-for-epicardial-defibrillator-lead-placement-in-young-children-coil-between-the-great-arteries
#13
Raghav Murthy, Matthew R Williams, James C Perry, Suzanne Shepard, Daniel DiBardino
The primary and secondary prevention of sudden cardiac death resulting from malignant arrhythmia, channelopathy, and hypertrophic cardiomyopathy often requires the implantation of automatic internal cardiac defibrillators (AICDs) in the pediatric population. In young patients, the small size of the systemic veins, complex anatomy of congenital heart disease, and body habitus often preclude safe and durable transvenous placement of the AICD coil, requiring innovative methods to circumvent this problem. This report describes the technique used at Rady Children's Hospital San Diego/UCSD for the epicardial placement of an ICD system with a transvenous ICD coil placed between the aorta and pulmonary artery, thereby producing a stable location and excellent coil-to-can vector for successful defibrillation...
October 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27590665/evolution-of-hypertrophic-cardiomyopathy-in-sarcomere-mutation-carriers
#14
Carolyn Y Ho, Allison L Cirino, Neal K Lakdawala, John Groarke, Anne Marie Valente, Christopher Semsarian, Steven D Colan, E John Orav
OBJECTIVE: The early natural history of sarcomere mutations and the evolution to hypertrophic cardiomyopathy (HCM) are poorly characterised. To describe phenotypic progression, we compared mutation carriers who developed HCM to those who did not during prospective longitudinal investigation. METHODS: Sarcomere mutation carriers without baseline left ventricular hypertrophy (LVH) were studied during participation in a pilot clinical trial testing diltiazem versus placebo...
September 2, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27545012/tissue-doppler-imaging-during-exercise-stress-echocardiography-demonstrates-a-mechanism-for-impaired-exercise-performance-in-children-with-hypertrophic-cardiomyopathy
#15
Matthew Eric Ferguson, Ritu Sachdeva, Scott E Gillespie, Gemma Morrow, William Border
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is associated with decreased exercise tolerance in children, presumably due to diastolic dysfunction. Modern imaging techniques to assess myocardial function during active exercise have not been applied to this population. We hypothesized that impaired contractile reserve, as assessed by tissue Doppler imaging (TDI) and strain, contributes to reduced exercise capacity in affected individuals. METHODS: Children (<18 years) with HCM and healthy age- and sex-matched controls were prospectively enrolled...
November 2016: Echocardiography
https://www.readbyqxmd.com/read/27504452/mitochondrial-cardiomyopathies
#16
REVIEW
Ayman W El-Hattab, Fernando Scaglia
Mitochondria are found in all nucleated human cells and perform various essential functions, including the generation of cellular energy. Mitochondria are under dual genome control. Only a small fraction of their proteins are encoded by mitochondrial DNA (mtDNA), whereas more than 99% of them are encoded by nuclear DNA (nDNA). Mutations in mtDNA or mitochondria-related nDNA genes result in mitochondrial dysfunction leading to insufficient energy production required to meet the needs for various organs, particularly those with high energy requirements, including the central nervous system, skeletal and cardiac muscles, kidneys, liver, and endocrine system...
2016: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27502104/clinical-presentation-of-pediatric-patients-at-risk-for-sudden-cardiac-arrest
#17
Aarti Dalal, Richard J Czosek, Joshua Kovach, Johannes C von Alvensleben, Santiago Valdes, Susan P Etheridge, Michael J Ackerman, Debbie Auld, Jeryl Huckaby, Courtney McCracken, Robert Campbell
OBJECTIVES: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). STUDY DESIGN: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014...
October 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27437663/how-hypertrophic-cardiomyopathy-became-a-contemporary-treatable-genetic-disease-with-low-mortality-shaped-by-50-years-of-clinical-research-and-practice
#18
Barry J Maron, Ethan J Rowin, Susan A Casey, Martin S Maron
Hypertrophic cardiomyopathy (HCM) is a relatively common genetic heart disease encumbered throughout much of its almost 60-year history by a large measure of misunderstanding and the perception of a grim outcome without effective treatment options. However, it is now apparent that the majority of patients affected with HCM can achieve normal or near-normal life expectancy without disability, and usually do not require major treatment interventions. Nevertheless, for those patients with HCM who are at risk for (or experience) disease-related complications, a constellation of comprehensive nonpharmacologic management strategies have evolved over the last 15 years, altering the natural history and disease course for many, including implantable defibrillators, heart transplant, external defibrillation/therapeutic hypothermia, advances in surgical myectomy, and alcohol ablation...
April 1, 2016: JAMA Cardiology
https://www.readbyqxmd.com/read/27373729/a-long-term-follow-up-study-of-carriers-of-hypertrophic-cardiomyopathy-mutations
#19
Don R McTaggart, Kathryn J Ogden, Jessica A Marathe
BACKGROUND: Adults who test positive for a mutation associated with the development of hypertrophic cardiomyopathy (HCM) but who have not manifested left ventricular hypertrophy (LVH) at the time of that diagnosis are now commonly identified in the era of genetic testing. There are little published data, however, on the long-term outlook for these phenotypically normal gene carriers. METHODS: Fifteen genotype positive/LVH negative patients with HCM were identified, seven of which were children when first diagnosed as gene carriers...
January 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/27353558/minimal-access-posterior-approach-for-extrapleural-thoracic-sympathectomy-a-cadaveric-study-and-cases
#20
Jeffrey S Raskin, Jesse J Liu, Hai Sun, Andrew Nemecek, Seshadri Balaji, Ahmed M Raslan
OBJECTIVE: Operatively, video-assisted thoracoscopic sympathectomy (VATS) involves pleural entry and poses risk in small children and patients with pulmonary disease. A conventional posterior sympathectomy is more invasive than VATS. We investigated a cadaveric feasibility study of a minimal access posterior approach for endoscopic extrapleural sympathectomy and discuss this minimal approach in children with cardiac sympathectomy. METHODS: A posterior endoscopic extrapleural approach for thoracic sympathectomy was performed using lightly embalmed cadavers; surgical corridor depth, width, and associated pleural violation were recorded...
September 2016: World Neurosurgery
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