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Pediatric cardiomyopathy

Cheryl L Cox, M Robyn Andersen, Aimee K Santucci, Les L Robison, Melissa M Hudson
PURPOSE/OBJECTIVES: To document the per survivor and per additional survivor screening costs of a mailed survivorship care plan (SCP) with advanced practice nurse (APN) telephone counseling (SCP+C) or without APN telephone counseling (SCP).
. DESIGN: Randomized, longitudinal clinical trial.
. SETTING: St. Jude Children's Research Hospital in Memphis, Tennessee.
. SAMPLE: 411 at-risk pediatric cancer survivors (aged 26-59 years), stratified by age (younger than 30 years versus 30 years or older), recommended screening frequency (every one, two, or five years), gender, and cancer diagnosis (hematologic versus solid tumor)...
November 1, 2016: Oncology Nursing Forum
Jörg Michel, Michael Hofbeck, Gina Spiller, Hanna Renk, Matthias Kumpf, Felix Neunhoeffer
INTRODUCTION: Data are still lacking about the use of terlipressin or vasopressin in the treatment of pediatric patients who are in a state of therapy-refractory shock. OBJECTIVE: The aim of this study was to evaluate the effect of terlipressin on hemodynamics in children with distributive shock and to describe any severe side effects. METHODS: Consecutive patients (n = 20) with catecholamine-resistant distributive shock who were treated with terlipressin were retrospectively enrolled in this study...
October 20, 2016: Paediatric Drugs
Suma P Goudar, G Hamilton Baker, Shahryar M Chowdhury, Kimberly J Reid, Girish Shirali, Mark A Scheurer
BACKGROUND: Adult studies demonstrate that echocardiographic measurements of cardiac function using speckle tracking correlate with invasive measurements, but such data in the pediatric population are sparse. Our aim was to compare speckle-derived measures of cardiac function to measurements routinely obtained by cardiac catheterization in children. METHODS: Echocardiograms were performed on the day of cardiac catheterization. Using Tomtec 2D Cardiac Performance Analysis, longitudinal strain (LS), longitudinal strain rate (LSR), early diastolic LSR, and ejection fraction (EF) for the right and left ventricle (RV and LV) were calculated via speckle tracking...
October 14, 2016: Echocardiography
Saro H Armenian, Melissa M Hudson, Ming Hui Chen, Steven D Colan, Lanie Lindenfeld, George Mills, Aida Siyahian, Sarah Gelehrter, Ha Dang, Wendy Hein, Daniel M Green, Leslie L Robison, F Lennie Wong, Pamela S Douglas, Smita Bhatia
BACKGROUND: Anthracyclines are widely used in the treatment of childhood cancer. One of the well-recognized side-effects of anthracycline therapy is dose-dependent cardiomyopathy that may progress to heart failure (HF) years after completion of cancer-directed therapy. This study will evaluate the efficacy of low-dose beta-blocker (carvedilol) for HF risk reduction in childhood cancer survivors at highest risk for HF. The proposed intervention has the potential to significantly reduce chronic cardiac injury via interruption of neurohormonal systems responsible for left ventricular (LV) remodeling, resulting in improved cardiac function and decreased risk of HF...
October 4, 2016: BMC Cardiovascular Disorders
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
Bhavika J Patel, Lillian Lai, Gary Goldfield, Renee Sananes, Patricia E Longmuir
Psychosocial health issues are common among children with cardiac diagnoses. Understanding parent and child perceptions is important because parents are the primary health information source. Significant discrepancies have been documented between parent/child quality-of-life data but have not been examined among psychosocial diagnostic instruments. This study examined agreement and discrepancies between parent and child reports of psychosocial health and quality of life in the paediatric cardiology population...
September 20, 2016: Cardiology in the Young
Hala Mahmoud Koura, Neamat M Abdalla, Mona Hamed Ibrahim, Maha M A Abo Hashish, Sherif Mohamed Zaki
BACKGROUND: B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially in the pediatric age group. OBJECTIVES: The aim of the study was to compare the changes of the NT-proBNP in pediatric patients with LRS and DCM. Correlation between the levels of NT-proBNP and the echocardiographic parameters in both groups was determined...
June 2016: Iranian Journal of Pediatrics
Rafal Ploski, Malgorzata Rydzanicz, Tomasz M Ksiazczyk, Maria Franaszczyk, Agnieszka Pollak, Joanna Kosinska, Ewa Michalak, Piotr Stawinski, Lidia Ziolkowska, Zofia T Bilinska, Bozena Werner
Restrictive cardiomyopathy is a rare form of pediatric cardiac disease, for which the known genes include MYH7, TNNT2, TNNI3, ACTC1, and DES. We describe a pediatric proband with fatal restrictive cardiomyopathy associated with septal hypertrophy and compound heterozygosity for TNNC1 mutations (NM_003280: p.A8V [c.C23T] and p.D145E [c.C435A]). This association between restrictive cardiomyopathy and TNNC1 mutations was strengthened by prospective observations on the second pregnancy in the family which revealed, in the presence of the same TNNC1 genotype, prenatally diagnosed hypertrophic cardiomyopathy which evolved into restrictive cardiomyopathy, heart failure and death at the age of 9 months...
September 8, 2016: American Journal of Medical Genetics. Part A
Eyal Reinstein, Ana Gutierrez-Fernandez, Shay Tzur, Concetta Bormans, Shai Marcu, Einav Tayeb-Fligelman, Chana Vinkler, Annick Raas-Rothschild, Dana Irge, Meytal Landau, Mordechai Shohat, Xose S Puente, Doron M Behar, Carlos Lopez-Otın
In the vast majority of pediatric patients with dilated cardiomyopathy, the specific etiology is unknown. Studies on families with dilated cardiomyopathy have exemplified the role of genetic factors in cardiomyopathy etiology. In this study, we applied whole-exome sequencing to members of a non-consanguineous family affected by a previously unreported congenital dilated cardiomyopathy syndrome necessitating early-onset heart transplant. Exome analysis identified compound heterozygous variants in the FLNC gene...
September 7, 2016: European Journal of Human Genetics: EJHG
J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden
Long-chain fatty acid oxidation disorders (LC-FAOD) can cause cardiac hypertrophy and cardiomyopathy, often presenting in infancy, typically leading to death or heart transplant despite ongoing treatment. Previous data on triheptanoin treatment of cardiomyopathy in LC-FAOD suggested a clinical benefit on heart function during acute failure. An additional series of LC-FAOD patients with critical emergencies associated with cardiomyopathy was treated with triheptanoin under emergency treatment or compassionate use protocols...
August 27, 2016: Molecular Genetics and Metabolism
Yan Wang, Ling Niu, Xiuhua He, Ying Xue, Nan Ling, Zhenzhou Wang, Xinjiang An
Cardiomyopathy is a heterogeneous heart disease. Although morbidity of pediatric cardiomyopathy has been on the increase, effective treatments have not been identified. The aim of the study was to examine the expression of ACR1 gene products in association with cardiomyopathy in children. In total, 73 patients and 76 healthy subjects were enrolled in the study, from April, 2013 to April, 2015. The relative expression of ACR1 mRNA and protein were quantified in all cases, using reverse transcription-quantitative polymerase chain reaction (RT-qPCR), ELISA and western blot analysis...
September 2016: Experimental and Therapeutic Medicine
Valentina Favalli, Eliana Disabella, Mariadelfina Molinaro, Marilena Tagliani, Anna Scarabotto, Alessandra Serio, Maurizia Grasso, Nupoor Narula, Carmela Giorgianni, Clelia Caspani, Monica Concardi, Manuela Agozzino, Calogero Giordano, Alexandra Smirnova, Takahide Kodama, Lorenzo Giuliani, Elena Antoniazzi, Riccardo G Borroni, Camilla Vassallo, Filippo Mangione, Laura Scelsi, Stefano Ghio, Carlo Pellegrini, Marialuisa Zedde, Laura Fancellu, GianPietro Sechi, Antonello Ganau, Stefania Piga, Annarita Colucci, Daniela Concolino, Maria Teresa Di Mascio, Danilo Toni, Marina Diomedi, Claudio Rapezzi, Elena Biagini, Massimiliano Marini, Maurizia Rasura, Maurizio Melis, Antonia Nucera, Donata Guidetti, Michelangelo Mancuso, Umberto Scoditti, Pamela Cassini, Jagat Narula, Luigi Tavazzi, Eloisa Arbustini
BACKGROUND: Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease, caused by defects of the alpha-galactosidase A (GLA) gene. AFD can affect the heart, brain, kidney, eye, skin, peripheral nerves, and gastrointestinal tract. Cardiology (hypertrophic cardiomyopathy), neurology (cryptogenic stroke), and nephrology (end-stage renal failure) screening studies suggest the prevalence of GLA variants is 0.62%, with diagnosis confirmation in 0.12%. OBJECTIVES: This study sought to expand screening from these settings to include ophthalmology, dermatology, gastroenterology, internal medicine, pediatrics, and medical genetics to increase diagnostic yield and comprehensively evaluate organ involvement in AFD patients...
September 6, 2016: Journal of the American College of Cardiology
RoseAnn L Scheller, Laurie Johnson, Angela Lorts, Thomas D Ryan
Sudden cardiac arrest (SCA) in the pediatric population is a rare and potentially devastating occurrence. An understanding of the differential diagnosis for the etiology of the cardiac arrest allows for the most effective emergency care and provides the patient with the best possible outcome. Pediatric SCA can occur with or without prodromal symptoms and may occur during exercise or rest. The most common cause is arrhythmia secondary to an underlying channelopathy, cardiomyopathy, or myocarditis. After stabilization, evaluation should include electrocardiogram, chest radiograph, and echocardiogram...
September 2016: Pediatric Emergency Care
A Sinha, O Lewis, R Kumar, S L H Yeruva, B H Curry
Attention-deficit/hyperactivity disorder (ADHD) is a chronic neurobiological disorder exhibited by difficulty maintaining attention, as well as hyperactivity and impulsive behavior. Central nervous system (CNS) stimulants are the first line of treatment for ADHD. With the increase in number of adults on CNS stimulants, the question that arises is how well do we understand the long-term cardiovascular effects of these drugs. There has been increasing concern that adults with ADHD are at greater risk for developing adverse cardiovascular events such as sudden death, myocardial infarction, and stroke as compared to pediatric population...
2016: Case Reports in Cardiology
Joyce T Johnson, Joshua D Robinson, Jie Deng, Cynthia K Rigsby
BACKGROUND: A comprehensive cardiac magnetic resonance (cardiac MR) study including both late gadolinium enhancement (LGE) and MR angiography may be indicated for patients with a history of acquired or congenital heart disease. OBJECTIVE: To study the novel use of an extracellular agent for assessment of LGE combined with a blood pool contrast agent for detailed MR angiography evaluation to yield a comprehensive cardiac MR study in these patients. MATERIALS AND METHODS: We reviewed clinical cardiac MR studies utilizing extracellular and blood pool contrast agents and noted demographics, clinical data and adverse events...
August 30, 2016: Pediatric Radiology
Tyler H Harris, Jeffrey G Gossett
Care of adults with coronary artery disease focuses on troponins to rapidly move patients to catheterization. Troponins are increasingly drawn in children, but emergent catheterization may not be indicted. We sought to establish etiologies of troponin elevation and ascertain the yield of diagnostic tests, in this population. Retrospective review of patients from January 1, 2002, to December 31, 2011, who had any elevated troponin during the study period. Patients were excluded for recent cardiac surgery, "significant" congenital heart disease, if they were neonates in the NICU or were on ECMO...
August 29, 2016: Pediatric Cardiology
Hak Ju Kim, Sungkyu Cho, Woong-Han Kim
Cardiac resynchronization therapy (CRT) is a new treatment for refractory heart failure. However, most patients with heart failure treated with CRT are adults, middle-aged or older with idiopathic or ischemic dilated cardiomyopathy. We treated a 12-year-old boy, who was transferred after cardiac arrest, with dilated cardiomyopathy, left bundle-branch block, and ventricular tachycardia. We performed cardiac resynchronization therapy with a defibrillator (CRT-D). After CRT-D, left ventricular ejection fraction improved from 22% to 44% assessed by echocardiogram 1 year postoperatively...
August 2016: Korean Journal of Thoracic and Cardiovascular Surgery
Jeremy P Moore, Shuo Wang, Erin L Albers, Jack C Salerno, Elizabeth A Stephenson, Maully J Shah, Andreas Pflaumer, Richard J Czosek, Jason M Garnreiter, Kathryn Collins, Andrew L Papez, Shubhayan Sanatani, Nicole B Cain, Prince J Kannankeril, James C Perry, Ravi Mandapati, Jennifer N A Silva, Seshadri Balaji, Kevin M Shannon
Tachycardia-induced cardiomyopathy (TIC) is a treatable cause of heart failure in children, but there is little information as to which clinical variables best discriminate TIC from other forms of cardiomyopathy. TIC cases with dilated cardiomyopathy (DC) from 16 participating centers were identified and compared with controls with other forms of DC. Presenting clinical, echocardiographic, and electrocardiographic characteristics were collected. Heart rate (HR) percentile was defined as HR/median HR for age, and PR index as the PR/RR interval...
October 1, 2016: American Journal of Cardiology
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
Timothy M Hoffman
OBJECTIVES: The objectives of this review are to discuss the paradigms used to stage heart failure in children, the classification and physiologic profile of cardiomyopathies, and the acute and chronic pharmacologic management of heart failure. DATA SOURCE: MEDLINE, PubMed. CONCLUSION: The etiology of chronic heart failure in pediatrics is vast. The paradigm of extrapolating adult clinical trials and technological advancements to treat heart failure in children has become a nonsustainable model...
August 2016: Pediatric Critical Care Medicine
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