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Pediatric Cardiology

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https://www.readbyqxmd.com/read/28642988/development-of-pulmonary-hypertension-during-treatment-with-diazoxide-a-case-series-and-literature-review
#1
Matthew R Timlin, Alexander B Black, Heather M Delaney, Renée I Matos, Candace S Percival
Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infancy. The mainstay of medical management for CHI is diazoxide. Diazoxide inhibits insulin release from the pancreas, but also causes smooth muscle relaxation and fluid retention so it is typically given with chlorothiazide. In July 2015, the FDA issued a drug safety communication warning that pulmonary hypertension (PH) had been reported in 11 infants being treated with diazoxide and that the PH resolved with withdrawal of diazoxide...
June 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28639151/central-arterial-function-measured-by-non-invasive-pulse-wave-analysis-is-abnormal-in-patients-with-duchenne-muscular-dystrophy
#2
Thomas D Ryan, John J Parent, Zhiqian Gao, Philip R Khoury, Elizabeth Dupont, Jennifer N Smith, Brenda Wong, Elaine M Urbina, John L Jefferies
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder caused by mutation of dystrophin. Cardiovascular involvement includes dilated cardiomyopathy. Non-invasive assessment of vascular function has not been evaluated in DMD. We hypothesize arterial wave reflection is abnormal in patients with DMD. Pulse wave analysis was performed on DMD patients with a SphygmoCor SCOR-PVx System to determine central blood pressure and augmentation index (AIx) as an assessment of arterial wave reflection. Results were compared to a control group...
June 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28639150/how-should-we-diagnose-persistence-of-the-artery-of-the-fifth-pharyngeal-arch
#3
Justin T Tretter, Eric J Crotty, Robert H Anderson, Michael D Taylor
No abstract text is available yet for this article.
June 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28639149/safety-feasibility-results-and-economic-impact-of-common-interventional-procedures-in-a-low-volume-region-of-the-united-states
#4
Aaron Clem, Sami Awadallah, Zahid Amin
The aim of the study was to evaluate the safety, feasibility, and economic benefit of a high-volume operator performing common interventional procedures in a rural region of the United States (U.S.). Rural areas of the U.S., even with well-equipped cardiac catheterization laboratories, may not have a full-time pediatric interventional cardiologist (PIC); this requires patients to travel out of state even for simple interventions. Since 2005, we have adopted a policy of performing cardiac catheterizations and common interventional procedures with a visiting PIC...
June 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28631208/pulmonary-vein-doppler-patterns-in-infants-with-single-right-ventricle-anomalies-after-initial-staged-palliations
#5
Edward C Kirkpatrick, Jessica Steltzer, Pippa Simpson, Amy Pan, Andrea Dragulescu, Christine B Falkensammer, Sarah Gelehrter, Wyman W Lai, Jami Levine, Stephen Miller, Thomas A Miller, Jay Pruetz, Ritu Sachdeva, Deepika Thacker, Peter Frommelt
The aim of this study was to describe serial changes in echocardiographic Doppler pulmonary vein flow (PVF) patterns in infants with single right ventricle (RV) anomalies enrolled in the Single Ventricle Reconstruction trial. Measurement of PVF peak systolic (S) and diastolic (D) velocities, velocity time integrals (VTI), S/D peak velocity and VTI ratios, and frequency of atrial reversal (Ar) waves were made at three postoperative time points in 261 infants: early post-Norwood, pre-stage II surgery, and 14 months...
June 19, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28631207/qrs-width-as-a-predictor-of-right-ventricular-remodeling-after-percutaneous-pulmonary-valve-implantation
#6
C Paech, I Dähnert, F T Riede, R Wagner, T Kister, K Nieschke, F Wagner, R A Gebauer
Recent data showed a right ventricular dyssynchrony in patients with tetralogy of Fallot (TOF). Percutaneous pulmonary valve implantation (PPVI) has become an important procedure to treat a pulmonary stenosis and/or regurgitation of the right ventricular outflow tract in these patients. Despite providing good results, there is still a considerable number of nonresponders to PPVI. The authors speculated that electrical dysfunction of the right ventricle plays an underestimated role in the outcome of patients after PPVI...
June 19, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28623384/the-role-of-cardiopulmonary-exercise-testing-for-decision-making-in-patients-with-repaired-tetralogy-of-fallot
#7
REVIEW
Frederic Dallaire, Rachel M Wald, Ariane Marelli
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. As a result of the surgical strategies employed at the time of initial repair, chronic pulmonary regurgitation (PR) is prevalent in this population. Despite sustained research efforts, patient selection and timing of pulmonary valve replacement (PVR) to address PR in young asymptomatic patients with repaired tetralogy of Fallot (rToF) remain a fundamental but as yet unanswered question in the field of congenital heart disease...
June 16, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28620754/variation-in-pediatric-post-ablation-care-a%C3%A2-survey-of-the-pediatric-and%C3%A2-congenital-electrophysiology-society-paces
#8
Brynn E Dechert, Macdonald Dick, David J Bradley, Martin J LaPage
Although catheter ablation is a standard treatment for pediatric arrhythmias, there are no consensus guidelines for follow-up care. This study describes the variation in post-ablation practices identified through a survey of the pediatric and congenital electrophysiology society (PACES). Pediatric and congenital electrophysiology society members were invited to participate in an online survey of post-ablation practices in September 2014. Survey questions targeted routine post-ablation practices for three common arrhythmia substrates: atrioventricular nodal reentry tachycardia, concealed accessory pathways (AP), and manifest APs...
June 15, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28616649/earlier-pulmonary-valve-replacement-in-down-syndrome-patients-following-tetralogy-of-fallot-repair
#9
Rachel T Sullivan, Peter C Frommelt, Garick D Hill
The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis...
June 14, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28612086/comparison-of-6-mm-versus-8-mm-tip-cryoablation-catheter-for-the-treatment-of-atrioventricular-nodal-reentrant-tachycardia-in-children-a-prospective-study
#10
Volkan Tuzcu, Enes Elvin Gul, Mehmet Karacan, Hacer Kamali, Nida Celik, Celal Akdeniz
Due to its safety profile, cryoablation (Cryo) for atrioventricular nodal reentrant tachycardia (AVNRT) is more commonly preferred over radiofrequency (RF) ablation in children in recent years. Recent studies demonstrated high long-term success rates comparable to radiofrequency ablation. The aim of this prospective study was to compare the efficacy and safety of an 8-mm-tip versus 6-mm-tip Cryo catheter in the treatment of AVNRT in children. A total of 125 consecutive patients over 10 years of age with AVNRT were included...
June 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28608149/exercise-capacity-and-self-efficacy-are-associated-with-moderate-to-vigorous-intensity-physical-activity-in-children-with-congenital-heart-disease
#11
Laura Banks, Shelly Rosenthal, Cedric Manlhiot, Chun-Po Steve Fan, Adam McKillop, Patricia E Longmuir, Brian W McCrindle
This study sought to determine whether exercise capacity, self-efficacy, and gross motor skills are associated with moderate-to-vigorous physical activity (MVPA) levels in children, and if these associations differ by congenital heart disease (CHD) type. Medical history was abstracted from chart review. We assessed MVPA levels (via accelerometry), percent-predicted peak oxygen consumption ([Formula: see text] cardiopulmonary exercise test), gross motor skill percentiles (test of gross motor development version-2), and self-efficacy [children's self-perceptions of adequacy and predilection for physical activity scale (CSAPPA scale)]...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28608148/hypoplastic-left-heart-syndrome-sequencing-reveals-a-novel-notch1-mutation-in-a-family-with-single-ventricle-defects
#12
Matthew D Durbin, Adrian G Cadar, Charles H Williams, Yan Guo, David P Bichell, Yan Ru Su, Charles C Hong
Hypoplastic left heart syndrome (HLHS) has been associated with germline mutations in 12 candidate genes and a recurrent somatic mutation in HAND1 gene. Using targeted and whole exome sequencing (WES) of heart tissue samples from HLHS patients, we sought to estimate the prevalence of somatic and germline mutations associated with HLHS. We performed Sanger sequencing of the HAND1 gene on 14 ventricular (9 LV and 5 RV) samples obtained from HLHS patients, and WES of 4 LV, 2 aortic, and 4 matched PBMC samples, analyzing for sequence discrepancy...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28608147/coarctation-index-predicts-recurrent-aortic-arch-obstruction-following-surgical-repair-of-coarctation-of-the-aorta-in-infants
#13
Gregory Adamson, Tara Karamlou, Phillip Moore, Luz Natal-Hernandez, Sarah Tabbutt, Shabnam Peyvandi
Recurrent aortic arch obstruction (RAAO) remains a major cause of morbidity following surgical neonatal repair of coarctation of the aorta (CoA). Elucidating predictors of RAAO can identify high-risk patients and guide postoperative management. The Coarctation index (CoA-I), defined as the ratio of the diameter of the narrowest aortic arch segment to the diameter of the descending aorta, has been used to help diagnose RAAO in neonates following the Norwood Procedure. We sought to assess the predictive value of the CoA-I on RAAO after CoA repair in infants with biventricular circulation...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28589408/critically-underdeveloped-left-heart-morphology-associated-with-prematurity-and-low-birth-weight-conditional-staged-rehabilitation-towards-biventricular-repair-and-time-related-growth-of-left-heart-structures
#14
Fareed Ahmad, Robert Mangano, Shirah Shore, Anastasios Polimenakos
This is a case report of premature low birth weight infant with hypoplasia of left heart structures and a large malaligned VSD who underwent successful staged approach of biventricular repair. We obtained qualitative and quantitative echocardiographic, MRI, and conventional catheterization data to support stepwise strategy towards LV rehabilitation to sustain adequate cardiac output. A thorough and intense follow-up has shown significant growth of left heart structures and favorable clinical status following staged biventricular repair...
June 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28589407/changes-of-cerebral-oxygenation-in-sequential-glenn-and-fontan-procedures-in-the-same-children
#15
Yasunori Yagi, Masataka Yamamoto, Hitoshi Saito, Toshihiro Mori, Yuji Morimoto, Takayoshi Oyasu, Tsuyoshi Tachibana, Yoichi M Ito
Recently, it is common to perform the Fontan procedure after the Glenn procedure as surgical repair for the univentricular heart. How the brain oxygen saturation (rSO2) values change with the cardiac restoration and the process of growth during these procedures in individual children remains unknown. In this study, we retrospectively studied rSO2 data as well as the perioperative clinical records of 30 children who underwent both Glenn and Fontan procedures by the same surgeon in the same institute. The rSO2 was measured at the beginning and end of each procedure with an INVOS 5100C...
June 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28589406/erratum-to-preprocedural-risk-assessment-prior-to-ppvi-with-cmr-and-cardiac-ct
#16
Ladonna Malone, Brian Fonseca, Thomas Fagan, Jane Gralla, Neil Wilson, Daniel Vargas, Micheal DiMaria, Uyen Truong, Lorna P Browne
No abstract text is available yet for this article.
June 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28589405/drug-coated-balloon-angioplasty-a-novel-treatment-for-pulmonary-artery-in-stent-stenosis-in-a-patient-with-williams-syndrome
#17
Jennifer L Cohen, Julie S Glickstein, Matthew A Crystal
A 20-month-old boy with Williams syndrome had undergone multiple surgical and catheter-based interventions for resistant peripheral pulmonary arterial stenoses with eventual bilateral stent placement and conventional balloon angioplasty. He persistently developed suprasystemic right ventricular (RV) pressure. Angioplasty with a drug-coated balloon (DCB) was performed for in-stent restenosis and to remodel his distal pulmonary vessels bilaterally. This resulted in immediate improvement in the in-stent stenosis and resultant decrease in RV pressure...
June 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28555404/abnormal-myocardial-contractility-after-pediatric-heart-transplantation-by-cardiac-mri
#18
Heynric B Grotenhuis, Emile C A Nyns, Paul F Kantor, Anne I Dipchand, Steven C Greenway, Shi-Joon Yoo, George Tomlinson, Rajiv R Chaturvedi, Lars Grosse-Wortmann
Acute cellular rejection (ACR) compromises graft function after heart transplantation (HTX). The purpose of this study was to describe systolic myocardial deformation in pediatric HTX and to determine whether it is impaired during ACR. Eighteen combined cardiac magnetic resonance imaging (CMR)/endomyocardial biopsy (EMBx) examinations were performed in 14 HTX patients (11 male, age 13.9 ± 4.7 years; 1.2 ± 1.3 years after HTX). Biventricular function and left ventricular (LV) circumferential strain, rotation, and torsion by myocardial tagging CMR were compared to 11 controls as well as between patients with and without clinically significant ACR...
May 30, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28553678/population-study-confirms-serum-proteins-change-and-reveals-diagnostic-values-in-congenital-ventricular-septal-defect
#19
Jinghua Long, Shun Liu, Xiaoyun Zeng, Xiaoyun Yang, Hui Huang, Yongbo Zhang, Jiehua Chen, Yang Xu, Dongping Huang, Xiaoqiang Qiu
This study was designed to validate thrombospondin 1 (TSP-1), vascular endothelial-cadherin complex (VE-cad), insulin-like growth factor 2 (IGF-2), and amyloid precursor protein (APP) and assess their diagnostic value in ventricular septal defect (VSD). We investigated the serum levels of TSP-1, VE-cad, IGF-2, and APP by enzyme-linked immunosorbent assay in a hospital-based case-control study that included 40 VSD children and 40 healthy controls. Logistic regression analysis was applied to evaluate the relationship of the proteins and VSD, and receiver operating characteristic (ROC) curve was used to assess the diagnostic value of the significant proteins...
May 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28551818/novel-three-dimensional-image-fusion-software-to-facilitate-guidance-of-complex-cardiac-catheterization-3d-image-fusion-for-interventions-in-chd
#20
Sebastian Goreczny, Pawel Dryzek, Gareth J Morgan, Maciej Lukaszewski, Jadwiga A Moll, Tomasz Moszura
We report initial experience with novel three-dimensional (3D) image fusion software for guidance of transcatheter interventions in congenital heart disease. Developments in fusion imaging have facilitated the integration of 3D roadmaps from computed tomography or magnetic resonance imaging datasets. The latest software allows live fusion of two-dimensional (2D) fluoroscopy with pre-registered 3D roadmaps. We reviewed all cardiac catheterizations guided with this software (Philips VesselNavigator). Pre-catheterization imaging and catheterization data were collected focusing on fusion of 3D roadmap, intervention guidance, contrast and radiation exposure...
May 27, 2017: Pediatric Cardiology
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