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Pediatric Cardiology

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https://www.readbyqxmd.com/read/28725922/genetic-testing-protocol-reduces-costs-and-increases-rate-of-genetic-diagnosis-in-infants-with-congenital-heart-disease
#1
Gabrielle C Geddes, Donald Basel, Peter Frommelt, Aaron Kinney, Michael Earing
Genetic testing is routinely performed on infants with critical congenital heart disease (CHD). This project reviewed the effect of implementing a genetic testing protocol in this population. Charts of infants with critical CHD were reviewed for genetic testing and results across two time periods: the time before implementation of a genetic testing protocol (pre-protocol) and the time after implementation (post-protocol). The use of karyotype, 22q11.2 Deletion testing, and chromosomal microarray were compared across these two time periods...
July 19, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28721547/the-effect-on-somatic-growth-of-surgical-and-catheter-treatment-of-secundum-atrial-septal-defects
#2
Meghan M Chlebowski, Hongying Dai, Stephen F Kaine
Historical studies suggest an association between atrial septal defect (ASD) and impaired growth with inconsistent improvement following closure. Limited data exist regarding the impact on growth in the era of transcatheter therapy. To evaluate the effect of closure on growth, we conducted a retrospective review of patients undergoing surgical or transcatheter closure during two time periods. Four hundred patients with isolated secundum ASD were divided into three cohorts: early surgical, contemporary surgical, and transcatheter...
July 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711967/fate-of-duct-dependent-discontinuous-pulmonary-arteries-after-arterial-duct-stenting
#3
Giuseppe Santoro, Giovanbattista Capozzi, Mario Giordano, Gianpiero Gaio, Maria Teresa Palladino, Carola Iacono, Heba Talat Mahmoud, Maria Giovanna Russo
Significant and balanced PA growth following arterial duct (AD) stenting has already been consistently reported in literature. However, to date, no data are available about the role of this approach as palliation of congenital heart disease with a duct-dependent discontinuous pulmonary artery (dPA). The aim of this study was to evaluate the fate of a dPA of ductal origin following trans-catheter AD stabilization. Angiographic PA evaluation was performed in seven patients submitted to neonatal AD stenting as palliative recruitment of dPA...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711966/modified-single-patch-technique-versus-two-patch-technique-for-the-repair-of-complete-atrioventricular-septal-defect-a-meta-analysis
#4
Dongxu Li, Qiang Fan, Tomoyuki Iwase, Yasutaka Hirata, Qi An
Technical selection for surgical repair of complete atrioventricular septal defect (CAVSD) still remains controversial. This meta-analysis aimed to compare the modified single-patch (MP) technique with the two-patch (TP) technique for patients with CAVSD. Relevant studies comparing the MP technique with the TP technique were identified through a literature search using MEDLINE, EMBASE, Google Scholar, Cochrane Library, and the China National Knowledge Infrastructure databases. The variables were ventricular septal defect (VSD) size, cardiopulmonary bypass (CBP) time, aortic cross-clamp (ACC) time, intensive care unit stay, hospital stay, and other outcomes involving mortality, left ventricular outflow tract obstruction, atrioventricular valve regurgitation, residual septal shunt, atrioventricular block, and reoperation...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711965/efficacy-of-an-extracellular-matrix-in-systemic-loading-conditions-in-congenital-heart-surgery
#5
Adeel Ashfaq, Amit Iyengar, Oh Jin Kwon, Mohammad S Soroya, Son Nguyen, Ryan Ou, Brian Reemtsen
Extracellular matrices (ECM) are commonly used to repair congenital heart defects; however, there is a lack of literature pertaining to outcomes with ECM use in high-pressure conditions. Between 2011 and 2014, a total of 202 patients underwent congenital heart disease repair using the ECM placed in a systemic pressure condition. The operative sites included: defects in the ventricular septum, mitral valve, aortic valve, ascending aorta, and aortic arch. Patients were followed and evaluated for mortality and reoperations due to loss of ECM integrity...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711964/utility-of-echocardiography-in-detecting-silent-complications-after-pediatric-catheter-ablations
#6
Shahnawaz M Amdani, Salaam Sallaam, Peter P Karpawich, Sanjeev Aggarwal
Although transcatheter arrhythmia ablation (TCA) has been performed in children for over two decades, guidelines for routine use of post-ablation transthoracic echocardiography (TTE) are absent. We sought to determine the efficacy of TTE after apparently uneventful TCA procedures in detecting adverse findings and identify predisposing factors. A retrospective review of clinical and procedural data on patients who underwent TCA for supraventricular arrhythmias from 2000 to 2015 was performed. Pre- and post-ablation TTE data were reviewed...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28711963/transfer-of-neonates-with-critical-congenital-heart-disease-within-a-regionalized-network
#7
Michael F Swartz, Jill M Cholette, Jennifer M Orie, Marshall L Jacobs, Jeffrey P Jacobs, George M Alfieris
Regionalization of pediatric cardiac surgical care varies between and within states. In most geographic regions, at least some neonates with critical heart disease are transferred from their birth hospital to a different hospital for surgery. The impact of neonatal transfer for surgery, particularly over a considerable distance (>10 miles), has been largely unexplored. We sought to examine the impact of transferring neonates for cardiac surgery. We queried the New York State Cardiac Surgery database (2005-2014) from a single institution to identify neonates born within the cardiac surgery center and those transferred for surgery...
July 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#8
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28702717/mindfulness-based-stress-reduction-and-group-support-decrease-stress-in-adolescents-with-cardiac-diagnoses-a-randomized-two-group-study
#9
Vicki A Freedenberg, Pamela S Hinds, Erika Friedmann
Adolescents with cardiac diagnoses face unique challenges that can cause psychosocial distress. This study compares a Mindfulness-Based Stress Reduction (MBSR) program to a video online support group for adolescents with cardiac diagnoses. MBSR is a structured psycho-educational program which includes yoga, meditation, cognitive restructuring, and group support. A published feasibility study by our group showed significant reduction in anxiety following this intervention. Participants were randomized to MBSR or video online support group, and completed measures of anxiety, depression, illness-related stress, and coping pre- and post-6-session interventions...
July 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28702716/can-the-pulmonary-artery-wedge-pressure-be-used-reliably-as-a-surrogate-for-the-left-atrial-mean-pressure-in-pre-fontan-evaluation
#10
Bassel Mohammad Nijres, Ra-Id Abdulla, Sawsan Awad, Joshua Murphy
The correlation between mean pulmonary artery wedge pressure (PAWP) and left atrial mean pressure (LAMP) has been poorly studied in patients with single ventricle (SV) physiology (Bernstein et al. in Pediatr Cardiol 33: 15-20 2012). The aim of this study is to determine if the PAWP can be used safely as a surrogate to the LAMP to calculate the pulmonary vascular resistance (PVR) during the pre-Fontan evaluation. Also, we aimed to understand if the presence of significant systemic-to-pulmonary collaterals (SPCs) is a confounding factor for accurate estimation of the LAMP...
July 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28695245/risk-factors-for-peri-procedural-arterial-ischaemic-stroke-in-children-with-cardiac-disease
#11
Hiroko Asakai, Belinda Stojanovski, John C Galati, Dianna Zannino, Michael Cardamone, Darren Hutchinson, Michael M H Cheung, Mark T Mackay
Improved survival of children with congenital heart disease has led to increasing focus on neurodevelopmental outcome, as close to half of the infants undergoing cardiac surgery are affected by neurodevelopmental disability. Stroke is particularly important as it frequently results in permanent neurologic sequelae. The aim of this study was to investigate risk factors for peri-procedural arterial ischaemic stroke (AIS) in children with cardiac disease. A retrospective case-control analysis of children aged <18 years with radiologically confirmed AIS following a cardiac procedure admitted to the Royal Children's Hospital Melbourne between 1993 and 2010...
July 11, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28689328/image-quality-of-coronary-arteries-on-non-electrocardiography-gated-high-pitch-dual-source-computed-tomography-in-children-with-congenital-heart-disease
#12
Yuichiro Kanie, Shuhei Sato, Akihiro Tada, Susumu Kanazawa
This study aimed to evaluate image quality of coronary artery imaging on non-electrocardiography (ECG)-gated high-pitch dual-source computed tomography (DSCT) in children with congenital heart disease (CHD) and to assess factors affecting image quality. We retrospectively reviewed the records of 142 children with CHD who underwent non-ECG-gated high-pitch DSCT. The subjective image quality of the proximal coronary segments was graded using a five-point scale. A score <3 represented a non-diagnostic image...
July 8, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28689327/in-utero-evidence-of-impaired-somatic-growth-in-hypoplastic-left-heart-syndrome
#13
Jourdan E Triebwasser, Marjorie C Treadwell
We hypothesized that fetuses with hypoplastic left heart syndrome (HLHS) have impaired growth compared to expected growth for gestational age. This is a retrospective cohort study including singleton fetuses with isolated HLHS identified from a single, referral center's ultrasound database. To account for variable timing of ultrasounds, z-scores for gestational age were assigned for each biometric parameter. We identified 169 fetuses, of which 96 had more than one ultrasound. The median number of ultrasound evaluations per fetus was 2 (range 1-5)...
July 8, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28687891/functional-analyses-of-a-novel-cited2-nonsynonymous-mutation-in-chinese-tibetan-patients-with-congenital-heart-disease
#14
Shiming Liu, Zhaobing Su, Sainan Tan, Bin Ni, Hong Pan, Beihong Liu, Jing Wang, Jianmin Xiao, Qiuhong Chen
CITED2 gene is an important cardiac transcription factor that plays a fundamental role in the formation and development of embryonic cardiovascular. Previous studies have showed that knock-out of CITED2 in mice might result in various cardiac malformations. However, the mechanisms of CITED2 mutation on congenital heart disease (CHD) in Chinese Tibetan population are still poorly understood. In the present study, 187 unrelated Tibetan patients with CHD and 200 unrelated Tibetan healthy controls were screened for variants in the CITED2 gene; we subsequently identified one potential disease-causing mutation p...
July 8, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28687889/normal-reference-ranges-for-pulmonary-artery-diameters-in-preterm-infants
#15
Lulu Abushaban, Mariappa Thinakar Vel, Jebaraj Rathinasamy, Prem N Sharma
To establish normal reference ranges and Z-scores for pulmonary artery diameters in preterm infants and their correlation with body surface area, body weight, and chronological age. In a prospective study, 268 preterm infants, who fulfilled the inclusion criteria were examined. Echocardiograms were performed to measure the main pulmonary artery, right pulmonary artery, and left pulmonary artery diameters on day(s) 0 to -6 of life and at weekly intervals until they reached 36 weeks of age. Body surface area was divided into thirteen groups from 0...
July 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28687888/application-of-appropriate-use-criteria-for-initial-transthoracic-echocardiography-in-an-academic-outpatient-pediatric-cardiology-program
#16
Raya Safa, Sanjeev Aggarwal, Amrit Misra, Daisuke Kobayashi
Transthoracic echocardiography (TTE) is a non-invasive diagnostic modality for children with suspected heart disease. The American College of Cardiology published Appropriate Use Criteria (AUC) for an initial outpatient pediatric TTE in 2014 to promote effective care and improve resource utilization. The objective was to determine the appropriateness of TTE per the published AUC in a single academic pediatric cardiology clinic as a baseline performance quality measure. The echocardiography database was used to identify initial outpatient TTE in children during January-March 2014...
July 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28681132/lower-hospital-charges-and-societal-costs-for-catheter-device-closure-of-atrial-septal-defects
#17
Jessica N Sanchez, Michael D Seckeler
Atrial septal defects (ASD) are among the most common congenital heart defects. As more ASDs are corrected by interventional catheterization instead of surgery, it is critical to understand the associated clinical and societal costs. The goal of this study was to use a national U.S. database to describe hospital charges and societal costs for surgical and catheter-based (ASD) closure. Retrospective review of hospital discharge data from the Kids' Inpatient Database from January 2010 to December 2012. The database was queried for admissions for <21 years old with ICD-9 procedure codes for surgical (35...
July 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28681131/hemodynamic-evaluation-of-children-with-persistent-or-recurrent-pulmonary-arterial-hypertension-following-complete-repair-of-congenital-heart-disease
#18
Heiner Latus, Inken Wagner, Stefan Ostermayer, Gunter Kerst, Joachim Kreuder, Dietmar Schranz, Christian Apitz
Persistent or recurrent pulmonary arterial hypertension (PAH) following complete surgical repair of congenital heart disease (CHD) represents one of the largest group of PAH associated with CHD (PAH-CHD) in recent registry studies and seems to have a particularly poor prognosis. However, little is known about this fourth clinical subclass of PAH-CHD, especially in children. The purpose of this study was to assess specific characteristics of invasive hemodynamics of this disease in children, including acute vasodilator testing (AVT) and pulmonary endothelial function (PEF) and to compare to patients with idiopathic PAH (IPAH), who usually present with a similar fatal clinical course...
July 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28676926/the-infant-with-aortic-arch-hypoplasia-and-small-left-heart-structures-echocardiographic-indices-of-mitral-and-aortic-hypoplasia-predicting-successful-biventricular-repair
#19
Jennifer M Plymale, Peter C Frommelt, Melodee Nugent, Pippa Simpson, James S Tweddell, Amanda J Shillingford
In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC)...
July 4, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28669108/novel-genetic-variants-in-bag3-and-tnnt2-in-a-swedish-family-with-a-history-of-dilated-cardiomyopathy-and-sudden-cardiac-death
#20
Eva Fernlund, A Wålinder Österberg, E Kuchinskaya, M Gustafsson, K Jansson, C Gunnarsson
Familial dilated cardiomyopathy is a rare cause of dilated cardiomyopathy (DCM), especially in childhood. Our aim was to describe the clinical course and the genetic variants in a family where the proband was a four-month-old infant presenting with respiratory problems due to DCM. In the family, there was a strong family history of DCM and sudden cardiac death in four generations. DNA was analyzed initially from the deceased girl using next-generation sequencing including 50 genes involved in cardiomyopathy...
July 1, 2017: Pediatric Cardiology
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