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Pediatric cardiomyopathy

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https://www.readbyqxmd.com/read/28325512/-sudden-death-of-a-young-man-as-late-sequelae-complicating-a-pediatric-disease-about-a-case
#1
Clémence Pierry, Camille Franchet, Lucile Tuchtan-Torrents, Céline Macon, Julia Torrents, Caroline Capuani, Marie-Dominique Piercecchi-Marti
In France, sudden death is responsible every year for 40,000 deaths. The most frequent etiology is cardiac disease. Atheromatous-related pathology is the most common etiology beyond 35, but cardiomyopathies and channelopathies are responsible for a significant number of deaths in young adults. Some acquired disorders can also cause sudden cardiac death. We report the case of a 17-year-old man who died suddenly after sport. Autopsy and pathological study found multiple giant coronary aneurysms. Thrombosis and fibrous scar of myocardial ischemic events were observed...
March 18, 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/28315942/forgotten-right-ventricle-in-pediatric-dilated-cardiomyopathy
#2
Hala Mounir Agha, Hossam Ibrahim, Inas Abd El Satar, Naglae Abd El Rahman, Doaa Abd El Aziz, Zeinab Salah, Sonia El Saeidi, Fatma Mostafa, Wael Attia, Mohamed Abd El Rahman, Gaser Abd El Mohsen
To evaluate the right ventricular (RV) function in relation to that of the left ventricle (LV) in patients with dilated cardiomyopathy (DCM). Echocardiographic examination was done using tissue Doppler imaging (TDI) and two-dimensional speckle tracking echocardiography (2D-STE) for 32 pediatric patients with DCM comparing them to another 32 normal matched controls. The global longitudinal strain (GLS) derived from 2D-STE was used to reflect the LV systolic function. Tricuspid annular plan systolic excursion (TAPSE) and the following RV TDI derived indexes: peak systolic velocity (S'), peak early diastolic velocity E', peak late diastolic velocity A', isovolumic acceleration (IVA) and myocardial performance index (MPI) were measured...
March 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28295963/a-multicenter-study-of-the-impella-device-for-mechanical-support-of-the-systemic-circulation-in-pediatric-and-adolescent-patients
#3
V Vivian Dimas, Brian H Morray, Dennis W Kim, Christopher Sd Almond, Shabana Shahanavaz, Sebastian C Tume, Lynn F Peng, Doff B McElhinney, Henri Justino
OBJECTIVES: The objective was to review the use of Impella devices (Abiomed Inc, Danvers, MA) for temporary circulatory support in pediatric and adolescent patients (age ≤ 21 yrs). BACKGROUND: Options for minimally invasive circulatory support in children are limited, and published data are confined to case reports and small case series. METHODS: This was a retrospective, multicenter review of Impella implants in pediatric and adolescent patients from 2009-15, using standardized data collection and INTERMACS definitions...
March 15, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28295041/exome-sequencing-identifies-primary-carnitine-deficiency-in-a-family-with-cardiomyopathy-and-sudden-death
#4
Najim Lahrouchi, Elisabeth M Lodder, Maria Mansouri, Rafik Tadros, Layla Zniber, Najlae Adadi, Sally-Ann B Clur, Karin Y van Spaendonck-Zwarts, Alex V Postma, Abdelaziz Sefiani, Ilham Ratbi, Connie R Bezzina
Pediatric cardiomyopathy is a rare but severe disease with high morbidity and mortality. The causes are poorly understood and can only be established in one-third of cases. Recent advances in genetic technologies, specifically next-generation sequencing, now allow for the detection of genetic causes of cardiomyopathy in a systematic and unbiased manner. This is particularly important given the large clinical variability among pediatric cardiomyopathy patients and the large number of genes (>100) implicated in the disorder...
March 15, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28290258/parent-reports-of-health-related-quality-of-life-and-heart-failure-severity-score-independently-predict-outcome-in-children-with-dilated-cardiomyopathy
#5
Susanna L den Boer, Sara J Baart, Marijke H van der Meulen, Gabriëlle G van Iperen, Ad P Backx, Arend D Ten Harkel, Lukas A Rammeloo, Gideon J du Marchie Sarvaas, Ronald B Tanke, Willem A Helbing, Elisabeth M Utens, Michiel Dalinghaus
BACKGROUND: Dilated cardiomyopathy in children causes heart failure and has a poor prognosis. Health-related quality of life in this patient group is unknown. Moreover, results may provide detailed information of parents' sense of their child's functioning. We hypothesised that health-related quality of life, as rated by parents, and the paediatric heart failure score, as assessed by physicians, have both predictive value on outcome. Methods and results In this prospective study, health-related quality of life was assessed by parent reports: the Infant Toddler Quality of Life questionnaire (0-4 years) or Child Health Questionnaire-Parent Form 50 (4-18 years) at 3-6-month intervals...
March 14, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#6
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/28260473/perioperative-venoarterial-extracorporeal-membrane-oxygenation-support-during-heart-transplant
#7
Ender Gedik, Funda Atar, Aycan Ozdemirkan, Aynur Camkiran Firat, Pinar Zeyneloglu, Atilla Sezgin, Arash Pirat
OBJECTIVES: Heart transplant is the only definitive treatment of end-stage heart failure. Venoarterial extracorporeal membrane oxygenation may be used as a bridge to heart transplant. This technique may be used after heart transplant for conditions refractory to medical treatment like primary graft failure. Previously, we reported our experience with patients who received extracorporeal support as a bridge to emergency heart transplant. In this study, we present our perioperative experience with heart transplants in which extracorporeal support was used...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28258649/the-cardiac-manifestations-of-inherited-metabolic-diseases-in-children
#8
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/28258593/hypertrophic-cardiomyopathy-in-youth-athletes-successful-screening-with-point-of-care-ultrasound-by-medical-students
#9
John C Fox, Shadi Lahham, Graciela Maldonado, Suzi Klaus, Bassil Aish, Lauren V Sylwanowicz, Justin Yanuck, Sean P Wilson, Mason Shieh, Craig L Anderson, Carter English, Ryan Mayer, Uthara R Mohan
OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is a life-threatening genetic cardiovascular disease that often goes undetected in young athletes. Neither history nor physical examination are reliable to identify those at risk. The objective of this study is to determine whether minimally trained medical student volunteers can use ultrasound to screen for HCM. METHODS: This was a prospective enrollment of young athletes performed at 12 area high schools and three area colleges, between May 2012 and August 2013...
March 4, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28251324/middle-cerebral-artery-aneurysm-rupture-in-a-neonate-with-interrupted-aortic-arch-case-report
#10
Joaquin Hidalgo, James Charles Dickerson, Brandon Burnsed, Ali Luqman, James Mason Shiflett
INTRODUCTION: Arterial cerebral aneurysms in the neonatal population are rare, and while the association of interrupted aortic arch and intracranial aneurysm has been reported in the adult and pediatric population (three cases each), to date, it has not been reported in the neonate. CASE REPORT: We report the case of a 26-day-old girl who presented with a generalized seizure 2 weeks after undergoing congenital heart surgery. Head CT revealed diffuse SAH with a 1...
March 1, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28248808/continuous-intravenous-milrinone-therapy-in-pediatric-outpatients
#11
Michelle Curley, Jill Liebers, Roy Maynard
Milrinone is a phosphodiesterase 3 inhibitor with both positive inotropic and vasodilator properties. Administered as a continuous infusion, milrinone is indicated for the short-term treatment of patients with acute decompensated heart failure. Despite limited data supporting long-term milrinone therapy in adults with congestive heart failure, children managed as outpatients may benefit from continuous milrinone as a treatment for cardiac dysfunction, as a destination therapy for cardiac transplant, or as palliative therapy for cardiomyopathy...
March 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/28243594/an-overview-and-evaluation-of-recent-machine-learning-imputation-methods-using-cardiac-imaging-data
#12
Yuzhe Liu, Vanathi Gopalakrishnan
Many clinical research datasets have a large percentage of missing values that directly impacts their usefulness in yielding high accuracy classifiers when used for training in supervised machine learning. While missing value imputation methods have been shown to work well with smaller percentages of missing values, their ability to impute sparse clinical research data can be problem specific. We previously attempted to learn quantitative guidelines for ordering cardiac magnetic resonance imaging during the evaluation for pediatric cardiomyopathy, but missing data significantly reduced our usable sample size...
March 2017: Data (Basel)
https://www.readbyqxmd.com/read/28220574/selective-use-of-the-electrocardiogram-in-pediatric-preparticipation-athletic-examinations-among-pediatric-primary-care-providers
#13
Bradley C Clark, Joshua M Hayman, Charles I Berul, Kristin M Burns, Jonathan R Kaltman
OBJECTIVES: Recent literature examining insurance administrative data suggests that a selective approach, with concurrent history and physical exam (H&P), for obtaining an electrocardiogram (ECG) as a part of a preparticipation examination (PPE) for pediatric athletes is commonly used in the primary care setting demonstrating a high rate of disease detection. We sought to understand practice patterns of providers with regard to usage of ECG as a part of PPE. METHODS: Utilizing an online survey, we queried primary care providers regarding their practice patterns, rationale, and concerns regarding use of ECGs as a part of the PPE...
February 21, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28214152/clinical-and-genetic-diagnosis-of-familial-hypertrophic-cardiomyopathy-results-in-pediatric-cardiology
#14
Bárbara Cardoso, Inês Gomes, Petra Loureiro, Conceição Trigo, Fátima Ferreira Pinto
INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is most often of autosomal dominant inheritance with incomplete penetrance and variable expression. The main purpose of family screening is to identify relatives with unrecognized HCM and to monitor those at risk for disease, in order to minimize complications and to assess risk of sudden cardiac death. The ESC and ACCF/AHA guidelines on the diagnosis and management of HCM recommend the screening of child relatives from the age of 10-12 years...
March 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28193717/differences-in-presentation-and-outcomes-between-children-with-familial-dilated-cardiomyopathy-and-children-with-idiopathic-dilated-cardiomyopathy-a-report-from-the-pediatric-cardiomyopathy-registry-study-group
#15
Paolo Rusconi, James D Wilkinson, Lynn A Sleeper, Minmin Lu, Gerald F Cox, Jeffrey A Towbin, Steven D Colan, Steven A Webber, Charles E Canter, Stephanie M Ware, Daphne T Hsu, Wendy K Chung, John L Jefferies, Christina Cordero, Steven E Lipshultz
BACKGROUND: Research comparing the survival of children with familial dilated cardiomyopathy (FDCM) to that of children with idiopathic dilated cardiomyopathy (IDCM) has produced conflicting results. METHODS AND RESULTS: We analyzed data from children with FDCM or IDCM using the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry. Compared to children with IDCM (n=647), children with FDCM (n=223) were older (mean 6.2 versus 4.5 years, P<0...
February 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28193509/pilot-study-analyzing-automated-ecg-screening-of-hypertrophic-cardiomyopathy
#16
Matthew Campbell, Xuefu Zhou, Chia Han, Hedayat Abrishami, Gregory Webster, Christina Y Miyake, Christopher T Sower, Jeffery B Anderson, Timothy K Knilans, Richard J Czosek
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is one of the leading causes of sudden cardiac death in athletes. However, preparticipation ECG screening has often been criticized for failing to meet cost-effectiveness thresholds, in part because of high false-positive rates and the cost of ECG screening itself. OBJECTIVE: The purpose of this study was to assess the testing characteristics of an automated ECG algorithm designed to screen for HCM in a multi-institutional pediatric cohort...
February 11, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28183324/identification-of-taz-mutations-in-pediatric-patients-with-cardiomyopathy-by-targeted-next-generation-sequencing-in-a-chinese-cohort
#17
Jian Wang, Ying Guo, Meirong Huang, Zhen Zhang, Junxue Zhu, Tingliang Liu, Lin Shi, Fen Li, Huimin Huang, Lijun Fu
BACKGROUND: Barth syndrome (BTHS) is a rare X-linked recessive disease characterized by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. Early diagnosis and appropriate treatment may improve the prognosis of this disease. The purpose of this study is to determine the role of targeted next-generation sequencing (NGS) in the early diagnosis of BTHS in children with cardiomyopathy. METHODS: During the period between 2012 and 2015, a gene panel-based NGS approach was used to search for potentially disease-causing genetic variants in all patients referred to our institution with a clinical diagnosis of primary cardiomyopathy...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28169967/a-pediatric-case-with-takotsubo-cardiomyopathy-and-neurogenic-pulmonary-edema-due-to-an-epidural-hemorrhage
#18
Yuki Nakamori, Noriko Miyazawa, Kenji Yoshitani, Shinichi Yamamoto
No abstract text is available yet for this article.
February 6, 2017: Journal of Neurosurgical Anesthesiology
https://www.readbyqxmd.com/read/28162930/a-randomized-study-of-autologous-bone-marrow-derived-stem-cells-in-pediatric-cardiomyopathy
#19
E Sian Pincott, Deborah Ridout, Margaret Brocklesby, Angus McEwan, Vivek Muthurangu, Michael Burch
BACKGROUND: Bone marrow mononuclear cell fraction has been used as therapy for dilated cardiomyopathy in adults. Although case series are reported, there are no randomized controlled studies in children. METHODS: We designed a randomized, crossover, controlled pilot study to determine safety and feasibility of intracoronary stem cell therapy in children. The primary safety end-point was freedom from death and transplantation or any complication that could be considered related to bone marrow injection or anesthesia (e...
January 5, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28146440/sudden-collapse-of-a-preschool-aged-child-on-the-playground
#20
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
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