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Hypertrophic cardiomyopathy in children

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https://www.readbyqxmd.com/read/27789109/myocardial-dimensions-in-children-with-hypertrophic-cardiomyopathy-a-comparison-between-echocardiography-and-cardiac-magnetic-resonance-imaging
#1
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...
July 15, 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
#2
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
#3
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
#4
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...
September 22, 2016: 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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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...
May 20, 2016: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/27353558/minimal-access-posterior-approach-for-extrapleural-thoracic-sympathectomy-a-cadaveric-study-and-cases
#12
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
https://www.readbyqxmd.com/read/27348999/spectrum-of-mybpc3-gene-mutations-in-patients-with-hypertrophic-cardiomyopathy-reporting-two-novel-mutations-from-north-west-of-iran
#13
Leila Emrahi, Mehrnoush Toufan Tabrizi, Jalal Gharehsouran, Seyyed Mojtaba Mohaddes Ardebili, Mehrdad Asghari Estiar
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the leading cause of sudden cardiac death (SCD) in children and young adults and is the most frequent genetically determined cardiovascular disease following autosomal dominant pattern of inheritance. A number of genes have been shown to be responsible for HCM including MYBPC3. Cmybc, the protein encoded by MYBPC3 is a sarcomeric thick filament protein that interacts with titin, myosin, and actin to control sarcomeric gathering. Mutations in the MYBPC3 gene have been found to be associated with a history of sudden cardiac death in HCM patients...
2016: Clinical Laboratory
https://www.readbyqxmd.com/read/27246458/lower-spatial-qrs-t-angle-rules-out-sustained-ventricular-arrhythmias-in-children-with-hypertrophic-cardiomyopathy
#14
Daniel Cortez, Nandita Sharma, Jean Cavanaugh, Froilan Tuozo, Gwendolyn Derk, Emily Lundberg, Keith Weiner, Nafiz Kiciman, Juan Alejos, Bruce Landeck, Jamil Aboulhosn, Shelley Miyamoto, Anthony C McCanta, Anjan S Batra
: Introduction The spatial peaks QRS-T angle accurately distinguishes children with hypertrophic cardiomyopathy from their healthy counterparts. The spatial peaks QRS-T angle is also useful in risk stratification for ventricular arrhythmias. We hypothesised that the spatial peaks QRS-T angle would be useful for the prediction of ventricular arrhythmias in hypertrophic cardiomyopathy patients under 23 years of age. METHODS: Corrected QT interval and spatial peaks QRS-T angles were retrospectively assessed in 133 paediatric hypertrophic cardiomyopathy patients (12...
June 1, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27160105/extended-septal-myectomy-for-hypertrophic-obstructive-cardiomyopathy-in-children-and-adolescents
#15
Haitao Xu, Jun Yan, Qiang Wang, Dianyuan Li, Hongwei Guo, Shoujun Li, Ju Wang, Song Lou, Qingdong Zeng
Extended septal myectomy for children and adolescents with hypertrophic obstructive cardiomyopathy (HOCM) is a challenging procedure, and related data are currently limited. Our study objective was to assess the early outcomes in children and adolescents with HOCM after extended septal myectomy. From October 2007 to August 2015, 40 consecutive patients with HOCM underwent transaortic extended septal myectomy in Fuwai Hospital, Beijing, China. Patients clinical data were analyzed retrospectively. Mean age at the time of operation was 11...
August 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27061026/mybpc3-hypertrophic-cardiomyopathy-can-be-detected-by-using-advanced-ecg-in-children-and-young-adults
#16
E Fernlund, P Liuba, J Carlson, P G Platonov, T T Schlegel
INTRODUCTION: The conventional ECG is commonly used to screen for hypertrophic cardiomyopathy (HCM), but up to 25% of adults and possibly larger percentages of children with HCM have no distinctive abnormalities on the conventional ECG, whereas 5 to 15% of healthy young athletes do. Recently, a 5-min resting advanced 12-lead ECG test ("A-ECG score") showed superiority to pooled criteria from the strictly conventional ECG in correctly identifying adult HCM. The purpose of this study was to evaluate whether in children and young adults, A-ECG scoring could detect echocardiographic HCM associated with the MYBPC3 genetic mutation with greater sensitivity than conventional ECG criteria and distinguish healthy young controls and athletes from persons with MYBPC3 HCM with greater specificity...
May 2016: Journal of Electrocardiology
https://www.readbyqxmd.com/read/27047289/progressive-left-ventricular-hypertrophy-after-heart-transplantation-insights-and-mechanisms-suggested-by-multimodal-images
#17
Karthik Ananthasubramaniam, Kiran Garikapati, Celeste T Williams
Immunosuppression is the typical measure to prevent rejection after heart transplantation. Although rejection is the usual cause of cardiac hypertrophy, numerous other factors warrant consideration. Calcineurin inhibitors rarely cause hypertrophic cardiomyopathy; the few relevant reports have described children after orthotopic kidney or liver transplantation. We present the case of a 73-year-old woman, an asymptomatic orthotopic heart transplantation patient, in whom chronic immunosuppression with prednisone and cyclosporine apparently caused a phenotype of hypertrophic cardiomyopathy...
February 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/27004712/subcutaneous-defibrillator-implantation-in-pediatric-patients
#18
İlker Ertuğrul, Tevfik Karagöz, Hakan Aykan, Işıl Yıldırım, Sema Özer, Heves Karagöz, Mustafa Yılmaz
OBJECTIVE: Although sudden cardiac death is rare in children, an intracardiac defibrillator system is indicated in children with various types of cardiomyopathy, primary electrical diseases, and after surgical repair of congenital heart defects. The use of transvenous defibrillator lead systems is limited in pediatric patients because of a small body size and/or limited vascular access. Subcutaneous array leads combined with an abdominally placed generator can enable implantation. METHOD: This is a retrospective study of 13 patients who underwent subcutaneous defibrillator implantation between September 2010 and March 2015...
November 18, 2015: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/26961572/abnormal-mitral-valve-dimensions-in-pediatric-patients-with-hypertrophic-cardiomyopathy
#19
Daryl Schantz, Lee Benson, Jonathan Windram, Derek Wong, Andreea Dragulescu, Shi-Joon Yoo, Luc Mertens, Mark Friedberg, Bahiyah Al Nafisi, Lars Grosse-Wortmann
The hearts of patients with hypertrophic cardiomyopathy (HCM) show structural abnormalities other than isolated wall thickening. Recently, adult HCM patients have been found to have longer mitral valve leaflets than control subjects. The aim of the current study was to assess whether children and adolescents with HCM have similar measureable differences in mitral valve leaflet dimensions when compared to a healthy control group. Clinical and echocardiographic data from 46 children with myocardial hypertrophy and a phenotype and/or genotype consistent with sarcomeric HCM were reviewed...
April 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/26936621/comprehensive-versus-targeted-genetic-testing-in-children-with-hypertrophic-cardiomyopathy
#20
Nathan D Bales, Nicole M Johnson, Daniel P Judge, Anne M Murphy
Hypertrophic cardiomyopathy (HCM) is a genetic disease of the sarcomere that can be found in both children and adults and is associated with many causative mutations. In children who are not the index case of HCM in their families, current recommendations call only for targeted genetic testing for familial mutations. However, clinical experience suggests that de novo mutations are possible, as are mutations inherited from apparently an unaffected parent. A chart review was conducted of all patients who received HCM genetic testing at Johns Hopkins from 2004 to 2013...
June 2016: Pediatric Cardiology
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