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Hypertrophic cardiomyopathy in children

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https://www.readbyqxmd.com/read/29203757/variant-of-myocardial-infarction-course-in-the-patient-with-left-ventricular-non-compaction
#1
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
#2
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...
November 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29063585/early-8-days-systemic-postnatal-corticosteroids-for-prevention-of-bronchopulmonary-dysplasia-in-preterm-infants
#3
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
#4
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) i.e. ICD insertion, myectomy, aborted SCD, transplantation or death, was assessed by Cox regression...
October 20, 2017: 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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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