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pediatric congenital heart diseases

Daniel Verdini, Daniel Vargas, Anderson Kuo, Brian Ghoshhajra, Phillip Kim, Horacio Murillo, Jacobo Kirsch, Michael Lane, Carlos Restrepo
PURPOSE: Coronary-pulmonary arterial fistulas (CPAFs) are rare coronary artery anomalies that have been described only in limited case reports. This study aims to evaluate the clinical presentation and imaging findings of CPAFs collected from 6 participating medical centers along with CPAFs reported in the literature, to discern any general trends present in CPAFs. MATERIALS AND METHODS: A total of 25 cases of CPAF diagnosed by coronary computed tomography angiography were collected across 6 participating institutions...
November 2016: Journal of Thoracic Imaging
H Kaemmerer, C Apitz, K Brockmeier, A Eicken, M Gorenflo, A Hager, F deHaan, M Huntgeburth, R Kozlik-Feldmann, O Miera, G P Diller
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for different forms of PH, and specifically address PH associated with congenital heart disease (CHD). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary...
October 2016: Deutsche Medizinische Wochenschrift
Christoph Preuss, Melanie Capredon, Florian Wünnemann, Philippe Chetaille, Andrea Prince, Beatrice Godard, Severine Leclerc, Nara Sobreira, Hua Ling, Philip Awadalla, Maryse Thibeault, Paul Khairy, Mark E Samuels, Gregor Andelfinger
Left-ventricular outflow tract obstructions (LVOTO) encompass a wide spectrum of phenotypically heterogeneous heart malformations which frequently cluster in families. We performed family based whole-exome and targeted re-sequencing on 182 individuals from 51 families with multiple affected members. Central to our approach is the family unit which serves as a reference to identify causal genotype-phenotype correlations. Screening a multitude of 10 overlapping phenotypes revealed disease associated and co-segregating variants in 12 families...
October 2016: PLoS Genetics
Abby Li, Daniel Y Wang, Krista L Lanctôt, Ian Mitchell, Bosco A Paes
BACKGROUND: Respiratory syncytial virus hospitalization (RSVH) rates in children less than <2 years of age with hemodynamically significant congenital heart disease (HSCHD) are 2-4 fold higher compared with healthy term infants. Pediatric recommendations differ as to whether palivizumab is beneficial beyond 1 year of age. The objective was to determine whether differences exist in respiratory-related illness hospitalization (RIH) and RSVH in HSCHD infants receiving palivizumab during the first year versus second year of life in the Canadian Registry of Palivizumab (CARESS)...
October 6, 2016: Pediatric Infectious Disease Journal
Ana L Neves, Tiago Henriques-Coelho, Adelino Leite-Moreira, José C Areias
OBJECTIVE: The aim of this article is to evaluate the clinical utility of brain natriuretic peptide in pediatric patients, examining the diagnostic value, management, and prognostic relevance, by critical assessment of the literature. DATA SOURCES: In December 2015, a literature search was performed (PubMed access to MEDLINE citations; and included these Medical Subject Headings and text terms for the key words: "brain natriuretic peptide," "amino-terminal pro-brain natriuretic peptide," "children," "neonate/s," "newborn/s," "infant/s," and "echocardiography...
September 30, 2016: Pediatric Critical Care Medicine
Kirsten C Odegard, Robert Vincent, Rahul G Baijal, Suanne M Daves, Robert G Gray, Alexander J Javois, Barry A Love, Philip Moore, David Nykanen, Lori Q Riegger, Scott G Walker, Elizabeth C Wilson
Current practice of sedation and anesthesia for patients undergoing pediatric and congenital cardiac catheterization laboratory (PCCCL) procedures is known to vary among institutions, a multi-society expert panel with representatives from the Congenital Heart Disease Council of the Society for Cardiovascular Angiography and Interventions, the Society for Pediatric Anesthesia and the Congenital Cardiac Anesthesia Society was convened to evaluate the types of sedation and personnel necessary for procedures performed in the PCCCL...
October 3, 2016: Anesthesia and Analgesia
Candice Torres de Melo Bezerra Cavalcante, Nayana Maria Gomes de Souza, Valdester Cavalcante Pinto, Klébia Magalhães Pereira Castello Branco, Ronald Guedes Pompeu, Andreia Consuelo de Oliveira Teles, Rodrigo Cardoso Cavalcante, Giselle Viana de Andrade
Introduction: Risk Adjustment for Congenital Heart Surgery 1 (RACHS-1) score is a simple model that can be easily applied and has been widely used for mortality comparison among pediatric cardiovascular services. It is based on the categorization of several surgical palliative or corrective procedures, which have similar mortality in the treatment of congenital heart disease. Objective: To analyze the in-hospital mortality in pediatric patients (<18 years) submitted to cardiac surgery for congenital heart disease based on RACHS-1 score, during a 12-year period...
May 2016: Brazilian Journal of Cardiovascular Surgery
Nagat S El-Shmaa, Doaa El Amrousy, Wael El Feky
OBJECTIVE: The objective of this study was to assess the effectiveness of pre-emptive dexmedetomidine versus amiodarone in preventing junctional ectopic tachycardia (JET) in pediatric cardiac surgery. DESIGN: This is a prospective, controlled study. SETTING: This study was carried out at a single university hospital. SUBJECTS AND METHODS: Ninety patients of both sexes, American Society of Anesthesiologists Physical Status II and III, age range from 2 to 18 years, and scheduled for elective cardiac surgery for congenital and acquired heart diseases were selected as the study participants...
October 2016: Annals of Cardiac Anaesthesia
Mindl M Messinger, Kimberly L Dinh, Erin J McDade, Brady S Moffett, Angus A Wilfong, Antonio G Cabrera
BACKGROUND: Advances in cardiac operations over the last few decades, including corrective operations in early life, have dramatically increased the survival of children with congenital heart disease. However, postoperative care has been associated with neurologic complications, with seizures being the most common manifestation. The primary objective of this study is to describe the outcomes in pediatric patients who received an antiepileptic drug (AED) post-cardiac surgery. METHOD: A retrospective cohort study was performed in all patients less than 18 years of age who received an AED in the cardiovascular intensive care unit at Texas Children's Hospital from June 2002 until June 2012...
July 2016: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
Ji Young Kim, Yu Rim Shin, Hae Keum Kil, Mi Ran Park, Jong Wha Lee
BACKGROUND Rotational thromboelastometry (ROTEM®) is a point-of-care test for coagulation, enabling physicians to make a swift decision. The aim of this investigation was to establish reference intervals of thromboelastometric evaluation for coagulation in pediatric patients with congenital heart diseases (CHD). MATERIAL AND METHODS As baseline data, 3 assays of ROTEM® (INTEM, EXTEM, and FIBTEM) were measured after anesthesia induction. ROTEM® parameters were clotting time (CT), amplitude at 10 min (A10), clot formation time (CFT), a angle, maximal clot firmness (MCF), clot lysis index at 60 min (LI60), and maximal clot elasticity (MCE)...
October 6, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Kavita Sharma, Poonam Thankavel
Congenital stenosis/atresia of a coronary artery is an exquisitely rare anomaly (Congenit Heart Dis, 2, 2007, 347) with increased risk of sudden death. Bilateral coronary obstruction is even more unusual but has been reported in conjunction with aortic valve disease, syphilis, and Takayasu's arteritis. To the best of our knowledge, obstruction of both coronaries in a pediatric patient has only been reported once (Ann Thorac Surg, 55, 1993, 1564). We present a patient with an intramural, anomalous aortic origin of the right coronary artery from the contralateral sinus (AAORCA) with near atresia of the left main coronary ostium...
October 4, 2016: Echocardiography
Bana Agha Nasser, Julinar Idris, Abdu Rahman Mesned, Tageldein Mohamad, Mohamed S Kabbani, Ali Alakfash
BACKGROUND: Outcomes of cardiopulmonary resuscitation (CPR) in children with congenital heart disease have improved and many children have survived after an in-hospital cardiac arrest. AIM: The purpose of this study is to determine predictors of poor outcome after CPR in critical children undergoing cardiac surgery. METHODS: We conducted a retrospective chart review and data analysis of all CPR records and charts of all postoperative cardiac children who had a cardiac arrest and required resuscitation from 2011 until 2015...
October 2016: Journal of the Saudi Heart Association
Jamie L Jackson, Gina M Gerardo, Curt J Daniels, Kathryn Vannatta
BACKGROUND: Disease-related stressors for survivors of congenital heart disease (CHD) have been qualitatively described but not quantified nor examined in relationship to important patient-reported outcomes (PROs). OBJECTIVE: The aims of this study are to (1) identify the types and degree of disease-related stress experienced by CHD survivors based on age, functional status, and sex, (2) examine differences in stress and PROs by age, functional status, and sex, and (3) determine the unique contribution of perceived stress to variability in PROs...
September 28, 2016: Journal of Cardiovascular Nursing
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
Natalie Behrle, Peter Dyke, Abdallah Dalabih
Motor vehicle accident is the most common cause of blunt cardiac injury (BCI) in children (85.3%) due to the height of the child in relation to proper restraints and the compliant pediatric rib cage (J Trauma. 1996;40:200-202). Trauma to the chest wall may lead to injury of the myocardium, resulting in myocardial contusion, ventricular septal defect (VSD), ventricular free wall rupture, or valve compromise (J Trauma. 1996;40; 200-202; Heart Lung. 2012;41:200-202; J Inj Violence Res. 2012;4:98-100). There are several proposed mechanisms for the formation of VSD after blunt chest trauma including rupture of ischemic myocardium related to the initial trauma and reopening of a spontaneously closed congenital VSD...
September 23, 2016: Pediatric Emergency Care
Giovanni Biglino, Claudio Capelli, Despina Koniordou, Di Robertshaw, Lindsay-Kay Leaver, Silvia Schievano, Andrew M Taylor, Jo Wray
BACKGROUND: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models. OBJECTIVES: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses' perspective; and to identify possible improvements to optimise their use for teaching...
September 26, 2016: Congenital Heart Disease
Patricia E Thomas, Scott L Macicek
BACKGROUND: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. METHODS: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD...
2016: Ochsner Journal
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
Nobuyasu Kato, Masaaki Yamagishi, Keiichi Kanda, Takako Miyazaki, Yoshinobu Maeda, Masashi Yamanami, Taiji Watanabe, Hitoshi Yaku
PURPOSE: The ideal material for pediatric pulmonary artery (PA) augmentation is autologous pericardium. However, its utility for multistaged operations is limited. In this study, we applied an in vivo tissue-engineered autologous Biotube graft to a patient with congenital heart disease for the first time. DESCRIPTION: For molds of the Biotubes, two silicone 19F drain tubes were embedded in the subcutaneous spaces of a 2-year-old girl with a diagnosis of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries during palliative surgical procedures...
October 2016: Annals of Thoracic Surgery
Lin Yang, Jian Zhuang, Meiping Huang, Changhong Liang, Hui Liu
BACKGROUND: Hybrid iterative reconstruction can reduce image noise and produce better image quality compared with filtered back-projection (FBP), but few reports describe optimization of the iteration level. OBJECTIVE: We optimized the iteration level of iDose(4) and evaluated image quality for pediatric cardiac CT angiography. MATERIALS AND METHODS: Children (n = 160) with congenital heart disease were enrolled and divided into full-dose (n = 84) and half-dose (n = 76) groups...
September 16, 2016: Pediatric Radiology
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