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Hypertrophic cardiomyopathy in children

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https://www.readbyqxmd.com/read/29622585/prevalence-and-progression-of-late-gadolinium-enhancement-in-children-and-adolescents-with-hypertrophic-cardiomyopathy
#1
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/29513203/disopyramide-use-in-infants-and-children-with-hypertrophic-cardiomyopathy
#2
Matthew J O'Connor, Kelley Miller, Robert E Shaddy, Kimberly Y Lin, Brian D Hanna, Chitra Ravishankar, Joseph W Rossano
Hypertrophic cardiomyopathy has a range of clinical severity in children. Treatment options are limited, mainly on account of small patient size. Disopyramide is a sodium channel blocker with negative inotropic properties that effectively reduces left ventricular outflow tract gradients in adults with hypertrophic cardiomyopathy, but its efficacy in children is uncertain. A retrospective chart review of patients ⩽21 years of age with hypertrophic cardiomyopathy at our institution and treated with disopyramide was performed...
April 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29468337/psychosocial-impact-of-a-positive-gene-result-for-asymptomatic-relatives-at-risk-of-hypertrophic-cardiomyopathy
#3
Carissa Bonner, Catherine Spinks, Christopher Semsarian, Alex Barratt, Jodie Ingles, Kirsten McCaffery
Families with a history of hypertrophic cardiomyopathy (HCM) may be offered genetic testing in addition to clinical surveillance. Asymptomatic family members who are gene positive (silent gene carriers) represent a new group of "patients" who may not develop HCM, with little evidence available to assist clinical management. This study explored experiences of HCM genetic testing to identify potential benefits and harms. Thirty-two individuals previously offered genetic testing for HCM were recruited...
February 22, 2018: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/29467047/myocardial-stress-perfusion-magnetic-resonance-in-children-with-hypertrophic-cardiomyopathy
#4
Lazaro E Hernandez
BACKGROUND: Microvascular dysfunction in hypertrophic cardiomyopathy has been associated with poor clinical outcome. Several studies have demonstrated a reduced perfusion reserve proportional to the magnitude of the hypertrophy. We investigated the utility of stress perfusion cardiac MRI to detect microvascular dysfunction in children with hypertrophic cardiomyopathy. METHODS: From January 2016 to January 2017, 13 patients, with a mean age of 15.3 years, with hypertrophic cardiomyopathy underwent regadenoson stress perfusion cardiac MRI (1...
February 22, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29459657/very-long-and-long-chain-3-hydroxy-acyl-coa-dehydrogenase-deficiency-correlates-with-deregulation-of-the-mitochondrial-fusion-fission-machinery
#5
Judith Hagenbuchner, Sabine Scholl-Buergi, Daniela Karall, Michael J Ausserlechner
Children diagnosed with Long-Chain-3-Hydroxy-Acyl-CoA-Dehydrogenase-Deficiency (LCHADD) or Very-Long-Chain-3-Hydroxy-Acyl-CoA-Dehydrogenase-Deficiency (VLCADD) frequently present with hypertrophic cardiomyopathy or muscle weakness which is caused by the accumulation of fatty acid metabolites due to inactivating mutations in the mitochondrial trifunctional protein. By analyzing mitochondrial morphology we uncovered that mutations within the HADHA or the ACADVL gene not only affect fatty acid oxidation, but also cause significant changes in the DNM1L/MFN2 ratio leading to the significant accumulation of truncated and punctate mitochondria in contrast to network-like mitochondrial morphology in controls...
February 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29408870/mid-term-outcomes-of-biventricular-obstruction-and-left-ventricular-outflow-tract-obstruction-after-surgery-correction-in-child-and-adolescent-patients-with-hypertrophic-cardiomyopathy
#6
Shanshan Zhai, Haitao Xu, Chaomei Fan, Yinjian Yang, Fei Hang, Xiying Guo, Hongyue Wang, Fujian Duan, Jun Yan
BACKGROUND: Data on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited. OBJECTIVE: Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection. METHODS: This retrospective study identified 21 BVOTO patients and recruited 27 LVOTO and 24 NO-HCM patients younger than 18 years presenting at our institution...
2018: PloS One
https://www.readbyqxmd.com/read/29309839/predictors-of-electrocardiographic-screening-failure-for-the-subcutaneous-implantable-cardioverter-defibrillator-in-children-a-prospective-multicenter-study
#7
Matthew Campbell, Jeremy P Moore, Narayanswami Sreeram, Johannes C von Alvensleben, Anjan Shah, Anjan Batra, Ian Law, Shubhayan Sanatani, Vincent Thomas, Farnoosh Nik-Ahd, Stephen Williams, Nina Nosavan, Jennifer Maldonado, Amelia Hart, Thuan Nguyen, Seshadri Balaji
BACKGROUND: Subcutaneous implantable cardioverter-defibrillator (SICD) shows promise for select patients at risk of sudden death. However, patients need to pass an electrocardiographic (ECG) screening (ECG-S) test before they can receive an SICD. Predictors of ECG-S failure in children are unclear. OBJECTIVE: The purpose of this study was to identify the incidence and predictive factors for failure of ECG-S in children. METHODS: Children 18 years and younger with a preexisting ICD underwent ECG-S for SICD...
January 5, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29203757/variant-of-myocardial-infarction-course-in-the-patient-with-left-ventricular-non-compaction
#8
Natalia Y Osovska, Oleksandr I Datsyuk, Natalia V Kuzminova, Yevhen V Shaprynskyi, Hennadii V Bevz, Yaroslav M Pashynskyy, Oleksandr D Bondarchuk, Olena V Sergiichuk, Yaroslav V Karyi, Galina M Mazur
Non-compacted left ventricle in adults is a rare occurrence, though it is diagnosed even more rarely. As a rule in patients with non-compacted left ventricle (LVNC) other pathologic condition is diagnosed, notably hypertrophic or dilated cardiomyopathy. The majority of LVNC cases are diagnosed in early infancy but currently there are asymptomatic cases detected by means of echocardiographic examination. Real prevalence of LVNC is unknown. According to many authors LVNC occurs in 9.2-9.5% of children with diagnosed cardiomyopathies...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29148123/donohue-syndrome-a-review-of-literature-case-series-and-anesthetic-considerations
#9
REVIEW
Alana Kirkwood, Grant Stuart, Louise Harding
BACKGROUND: Donohue syndrome is a rare autosomal recessive disorder of insulin resistance, causing a functional defect in insulin receptor function, and affecting the ability of the insulin to bind the receptor. Features include severe hyperinsulinism and fasting hypoglycemia, along with severe failure to thrive despite feeding. An accelerated fasting state results in muscle wasting, decreased subcutaneous fat, and an excess of thick skin. A reduced thoracic diameter is accentuated by increased abdominal distension, which impacts on respiratory reserve...
January 2018: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29063585/early-8-days-systemic-postnatal-corticosteroids-for-prevention-of-bronchopulmonary-dysplasia-in-preterm-infants
#10
REVIEW
Lex W Doyle, Jeanie L Cheong, Richard A Ehrenkranz, Henry L Halliday
BACKGROUND: Bronchopulmonary dysplasia remains a major problem in neonatal intensive care units. Persistent inflammation in the lungs is the most likely underlying pathogenesis. Corticosteroids have been used to prevent or treat bronchopulmonary dysplasia because of their potent anti-inflammatory effects. OBJECTIVES: To examine the relative benefits and adverse effects of systemic postnatal corticosteroids commenced within the first seven days of life for preterm infants at risk of developing bronchopulmonary dysplasia...
October 24, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29053178/utility-of-genetics-for-risk-stratification-in-pediatric-hypertrophic-cardiomyopathy
#11
J Mathew, L Zahavich, M Lafreniere-Roula, J Wilson, K George, L Benson, S Bowdin, S Mital
Children with hypertrophic cardiomyopathy (HCM) experience sudden cardiac death (SCD) and other life-threatening events. We assessed if affected gene and variant burden predict outcomes. Patients <18 years old with primary HCM with a pathogenic variant or variant of uncertain significance in cardiomyopathy genes were included. Association of gene and variant number and type with freedom from major adverse cardiac events (MACE), that is, ICD insertion, myectomy, aborted SCD, transplantation or death, was assessed by Cox regression...
February 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29046207/-clinical-characteristics-and-gaa-gene-mutation-in-children-with-glycogen-storage-disease-type-ii-an-analysis-of-3-cases
#12
Shan Yuan, Jie Jiang, Lu-Ting Zha, Zuo-Cheng Yang
Glycogen storage disease type II (GSD II) is an autosomal recessive disorder caused by a deficiency of the lysosomal glycogen-hydrolyzing enzyme acid α-glucosidase (GAA) and can affect multiple systems including the heart and skeletal muscle. The aim of this study was to investigate three children with GSD II confirmed by GAA gene analysis and to report their clinical characteristics and gene mutations. One case was classified as infantile-onset GSD II, and two cases as late-onset GSD II. The infantile-onset patient (aged 4 months) showed no weight increase and had dyspnea, muscle hypotonia, and increased alanine aminotransferase and creatine kinase; echocardiography showed hypertrophic cardiomyopathy...
October 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28958965/bnp-response-during-berlin-heart%C3%A2-support-in-child-a-case-report
#13
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/28944179/hypertrophic-cardiomyopathy-in-children
#14
Arman Arghami, Joseph A Dearani, Sameh M Said, Patrick W O'Leary, Hartzell V Schaff
Hypertrophic cardiomyopathy (HCM) occurs in 1 of 500 adults and is considered to be one of the most common causes of death in young people under 35 years of age. Children with HCM are usually asymptomatic and the overall annual mortality beyond the first year of life is 1%. Septal myectomy is safe and effective in children with obstructive HCM and published data shows improved late survival compared to untreated HCM. Patient selection and surgical expertise remain critical components to ensuring successful outcomes of septal myectomy, particularly when considering prophylactic myectomy in a seemingly asymptomatic patient...
July 2017: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#15
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
#16
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/28879059/pulmonary-hypertension-and-cardiac-hypertrophy-in-children-recipients-of-orthotopic-living-related-liver-transplantation
#17
Magd A Kotb, Inas Abd El Satar, Ahmed M Badr, Nancy H Anis, Hoda Abd El Rahman Ismail, Alaa F Hamza, Hesham M Abdelkader
Surgical stress, liberation of cytokines associated with re-perfusion injury, and long standing use of immune suppressive medications in children recipients of orthotopic living related liver transplantation (OLRLT) pose cardiovascular risk. Reported cardiovascular adverse effects vary from left ventricular wall thickening, hypertrophic cardiomyopathy to resting ECG abnormalities, asymptomatic ST depression following increased heart rate and ventricular arrhythmias. Twenty-five consecutive children recipients of OLRLT were assessed by conventional 2-D, M-mode echocardiography and Doppler...
November 2017: Journal of Advanced Research
https://www.readbyqxmd.com/read/28699125/uptake-of-predictive-genetic-testing-and-cardiac-evaluation-for-children-at-risk-for-an-inherited-arrhythmia-or-cardiomyopathy
#18
Susan Christian, Joseph Atallah, Robin Clegg, Michael Giuffre, Cathleen Huculak, Tara Dzwiniel, Jillian Parboosingh, Sherryl Taylor, Martin Somerville
Predictive genetic testing in minors should be considered when clinical intervention is available. Children who carry a pathogenic variant for an inherited arrhythmia or cardiomyopathy require regular cardiac screening and may be prescribed medication and/or be told to modify their physical activity. Medical genetics and pediatric cardiology charts were reviewed to identify factors associated with uptake of genetic testing and cardiac evaluation for children at risk for long QT syndrome, hypertrophic cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy...
July 11, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28688952/left-ventricular-mechanics-in-children-with-hypertrophic-cardiomyopathy-cmr-study
#19
Łukasz Mazurkiewicz, Lidia Ziółkowska, Joanna Petryka, Mateusz Śpiewak, Łukasz Małek, Agata Kubik, Magdalena Marczak, Jolanta Misko, Grażyna Brzezińska-Rajszys
OBJECTIVES: To assess the magnitude of myocardial displacement abnormalities and their alterations with the fibrosis, left-ventricular (LV) outflow tract obstruction (LVOTO) and hypertrophy in juveniles with hypertrophic cardiomyopathy (HCM). STUDY DESIGN: Fifty-five children [age 12,5±4.6years, 38 (69,1%) males, 19 (34,5%) with LVOTO] with HCM and 20 controls underwent cardiovascular magnetic resonance. The LV feature tracking (FT) derived strain and strain rates were quantified...
November 2017: Magnetic Resonance Imaging
https://www.readbyqxmd.com/read/28606196/prescribing-an-automated-external-defibrillator-for-children-at-increased-risk-of-sudden-arrhythmic-death
#20
Karen A McLeod, Eileen Fern, Fiona Clements, Ruth McGowan
BACKGROUND: Automated external defibrillators can be life-saving in out-of-hospital cardiac arrest. OBJECTIVE: Our aim was to review our experience of prescribing automated external defibrillators for children at increased risk of sudden arrhythmic death. METHODS: We reviewed all automated external defibrillators issued by the Scottish Paediatric Cardiac Electrophysiology Service from 2005 to 2015. All parents were given resuscitation training according to the Paediatric Resuscitation Guidelines, including the use of the automated external defibrillator...
September 2017: Cardiology in the Young
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