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Hypertrophic cardiomyopathy in children

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https://www.readbyqxmd.com/read/28958965/bnp-response-during-berlin-heart-%C3%A2-support-in-child-a-case-report
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/28592009/-clinical-and-gene-mutation-analysis-of-three-children-with-late-onset-glycogen-storage-disease-type-%C3%A2-with-hypertrophic-cardiomyopathy
#9
J H Luo, W J Qiu, D Fang, J Ye, L S Han, H W Zhang, Y G Yu, L L Liang, X F Gu
Objective: To investigate the clinical and laboratory features of three children with late-onset type Ⅱ glycogen storage disease(GSD) who presented with hypertrophic cardiomyopathy and to analyze the effect of five mutations identified on the acid-α-glucosidase (GAA) activity and stability. Method: Three cases of children with muscle weakness were included in this study.GAA activity was analyzed in Dried Blood Spot of the patients.DNA was extracted from peripheral blood in all the patients and their parents and subjected to polymerase chain reaction and directly sequencing of GAA gene...
June 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28452637/incidence-and-etiology-of-sudden-cardiac-death-new-updates-for-athletic-departments
#10
REVIEW
Irfan M Asif, Kimberly G Harmon
CONTEXT: Sudden cardiac death (SCD) in a young athlete is a tragic event and is the leading medical cause of death in this population. The precise incidence of SCD in young athletes has been subject of debate, with studies reporting drastically different rates (1:917,000 athlete-years (AYs) to 1:3000 AYs) depending on the methodological design of the investigation or the targeted population. EVIDENCE ACQUISITION: A literature search was performed in PubMed using the terms: incidence, sudden cardiac death, sudden death, sudden cardiac arrest, etiology, pathology, registry, athlete, young, children, and adolescents...
May 2017: Sports Health
https://www.readbyqxmd.com/read/28441660/the-phenotype-and-outcome-of-infantile-cardiomyopathy-caused-by-a-homozygous-elac2-mutation
#11
Zarghuna M A Shinwari, Abdulrahman Almesned, Ali Alakhfash, Ahmad M Al-Rashdan, Eissa Faqeih, Zainab Al-Humaidi, Ahmed Alomrani, Malak Alghamdi, Dilek Colak, Abdullah Alwadai, Monther Rababh, Majid Al-Fayyadh, Zuhair N Al-Hassnan
OBJECTIVE: Cardiomyopathy (CMP) in children is a clinically and genetically heterogeneous group of disorders. Disease-associated mutations have been identified in more than 50 genes. Recently, mutations in the mitochondrial tRNA processing gene, ELAC2, were reported to be associated with the recessively inherited form of hypertrophic CMP (HCM). This study is aimed at describing the cardiac phenotype and outcome of ELAC2 mutation. METHODS: We performed whole exome sequencing followed by targeted mutation screening to identify the genetic etiology of severe infantile-onset CMP in 64 consanguineous Saudi families...
2017: Cardiology
https://www.readbyqxmd.com/read/28429690/-our-experience-in-the-diagnosis-and-treatment-of-postural-orthostatic-tachycardia-syndrome-vasovagal-syncope-and-inappropriate-sinus-tachycardia-in-children
#12
Sezen Ugan Atik, Reyhan Dedeoğlu, Aida Koka, Funda Öztunç
OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28421174/utility-of-cardiovascular-magnetic-resonance-derived-wave-intensity-analysis-as-a-marker-of-ventricular-function-in-children-with-heart-failure-and-normal-ejection-fraction
#13
Hopewell N Ntsinjana, Robin Chung, Paolo Ciliberti, Vivek Muthurangu, Silvia Schievano, Jan Marek, Kim H Parker, Andrew M Taylor, Giovanni Biglino
OBJECTIVE: This study sought to explore the diagnostic insight of cardiovascular magnetic resonance (CMR)-derived wave intensity analysis to better study systolic dysfunction in young patients with chronic diastolic dysfunction and preserved ejection fraction (EF), comparing it against other echocardiographic and CMR parameters. BACKGROUND: Evaluating systolic and diastolic dysfunctions in children is challenging, and a gold standard method is currently lacking...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28401251/delayed-myocardial-enhancement-in-pediatric-hypertrophic-cardiomyopathy-correlation-with-lv-function-echocardiography-and-demographic-parameters
#14
Sonia El Saiedi, Noha Hossam Behairy, Ahmed Kharabish, Reem Esmail, Zeinab Salah Seliem, Mervat Shafik, Wesam El Mozy
Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI)...
June 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28396031/penetrance-of-hypertrophic-cardiomyopathy-in-children-who-are-mutation-positive
#15
Alexa M C Vermeer, Sally-Ann B Clur, Nico A Blom, Arthur A M Wilde, Imke Christiaans
OBJECTIVES: To investigate the presence of hypertrophic cardiomyopathy (HCM) at first cardiac evaluation and during follow-up and cardiac events in predictively tested children who are mutation positive. STUDY DESIGN: The study included 119 predictively tested children who were mutation positive, with a mean age of 12.1 years. A family history and clinical variables from all cardiac evaluations after predictive genetic testing were recorded. Outcome measures were a clinical diagnosis of HCM, death, and cardiac events...
September 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28337834/retinal-dystrophy-in-two-boys-with-costello-syndrome-due-to-the-hras-p-gly13cys-mutation
#16
Mary Ella Pierpont, Mary Richards, W Keith Engel, Nancy J Mendelsohn, C Gail Summers
Features of Costello Syndrome, a systemic disorder caused by germline mutations in the proto-oncogene HRAS from the RAS/MAPK pathway, include failure-to-thrive, short stature, coarse facial features, cardiac defects including hypertrophic cardiomyopathy, intellectual disability, and predisposition to neoplasia. Two unrelated boys with Costello syndrome and an HRAS mutation (p.Gly13Cys) are presented with their ophthalmologic findings. Both had early symptoms of nystagmus, photophobia, and vision abnormalities...
May 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#17
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
#18
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...
September 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
#19
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/cardiac-manifestations-of-inherited-metabolic-disease-in-children
#20
REVIEW
David Fa Lloyd, Roshni Vara, Sujeev Mathur
Inborn errors of metabolism (IEM) are responsible for around 5% of all cases of cardiomyopathy (CM) and for 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic CM, 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. IEM 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 and left ventricular non-compaction...
May 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
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