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Sheetal R. Patel^1,2,*, David M. Kwiatkowski³, Adin-Cristian Andrei², Ankita Devareddy², Hangzhi Shi², Catherine D. Krawczeski⁴, Natalie Ebert⁵, Barbara J. Deal^1,2, Craig B. Langman^2,6, Bradley S. Marino^1,2
Congenital Heart Disease, Vol.15, No.4, pp. 181-195, 2020, DOI:10.32604/CHD.2020.012097 - 07 September 2020
Abstract Objectives: The Fontan operation has increased survival in patients with single-ventricle congenital heart defects. However, Fontan survivors are at risk of other organ dysfunctions, such as renal dysfunction (RD). The objectives of this study are to assess the prevalence of and potential risk factors for RD among Fontan survivors. Design, setting, and patients: We performed a two-center, cross-sectional study that included Fontan survivors evaluated in outpatient-clinics for routine follow up between 01/08-12/16. Outcome measures: The primary outcome was the presence of RD defined by estimated glomerular filtration rate (eGFR) of <90 ml/min/1.73 m² derived using the serum… More >

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CASE REPORT

Naveed Rabbani^1,*, Sarah Hofman DeYoung², Ronald L. Gibson², Jeffrey Conwell¹, Jason F. Deen¹
Congenital Heart Disease, Vol.15, No.4, pp. 197-201, 2020, DOI:10.32604/CHD.2020.011527 - 07 September 2020
Abstract Persistent left superior vena cava (PLSVC) is a relatively common congenital venous anomaly that typically drains into the coronary sinus without hemodynamic significance. Rarely a PLSVC drains directly into the left atrium, forming a right-to-left shunt that can result in hypoxemia and potential paradoxical embolism. We present the case of a 2-year-old medically complex child on chronic mechanical ventilation with eventual diagnosis of episodic hypoxemia due to a PLSVC draining into the left atrium with position-dependent venous flow. The lesion was identified with contrast echocardiography and cardiac MRI. Subsequent occlusion with a vascular plug resulted More >

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ARTICLE

Mohd Nizam Mat Bah^*, Mohd Hanafi Sapian, Hasliza Razak, Emieliyuza Yusnita Alias
Congenital Heart Disease, Vol.15, No.4, pp. 203-216, 2020, DOI:10.32604/CHD.2020.013057 - 07 September 2020
Abstract Introduction: Fetal echocardiogram allows early detection of critical congenital heart disease leading to a better outcome. However, data from lowerand middle-income countries is scarce. This study aims to evaluate the diagnostic error of fetal echocardiography and its impact on planned neonatal management. Methods and material: This retrospective observational cohort study includes all high-risk pregnant mothers who had fetal echocardiograms from 2008 to 2017. Fetal and postnatal echocardiograms were compared, while the diagnostic errors were categorized into false positive, false negative, and discrepant diagnoses. The impact of the diagnostic error on planned neonatal management and the long-term outcome… More >

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1748

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ARTICLE

Angeles Fuertes-Moure¹, Michael Meyer^2,3, Anna-Luisa Häcker^2,3, Barbara Reiner^2,3, Leon Brudy³, Sonia Pértega-Diaz⁴, Renate Oberhoffer^2,3, Peter Ewert², Jan Müller^2,3,*
Congenital Heart Disease, Vol.15, No.4, pp. 217-227, 2020, DOI:10.32604/CHD.2020.011771 - 07 September 2020
Abstract Objective: Health-related quality of life (HRQoL) has become an important outcome measure for patients with congenital heart disease (CHD). The aim of this study was to evaluate the natural course of HRQoL from longitudinal assessment in children with CHD. Patients and Methods: From July 2014 to February 2020 this longitudinal study recruited 317 children with CHD (113 girls, 35.6%) aged 6 to 18 years (11.6 ± 2.9 years). HRQoL was assessed with the generic, self-reported and age-adapted KINDL^® questionnaire. During a mean follow-up period of 2.2 ± 1.3 years, 195 patients had one HRQoL reassessment, 70 two,… More >

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1651

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ARTICLE

Carter Biewen^1,*, Hitendra Patel², Olaf Reinhartz³, Ginny Gildengorin⁴, Natalie Cvijanovich⁵
Congenital Heart Disease, Vol.15, No.4, pp. 229-238, 2020, DOI:10.32604/CHD.2020.011671 - 07 September 2020
Abstract Purpose: To summarize Fontan procedure data from our institution, UCSF Benioff Children’s Hospital Oakland, in order to better understand outcomes of our surgical and post-operative care. Basic Procedures: This is a retrospective cross-sectional chart review summarizing characteristics and outcomes of patients who underwent the Fontan procedure at our institution between 2005 and 2016. Main Findings: Sixty-five Fontan procedures were performed at our institution between 2005 and 2016, all of which were extracardiac Fontan procedures performed by the same surgical team. At the time of Fontan procedure, the mean patient age was 56.4 ± 18.7 months and mean weight… More >

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1434

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Or Bercovich¹, Tal Tirosh-Wagner², Lior Goldberg¹, Amir Vardi³, David Mishali⁴, Gideon Paret^1,#, Yael Nevo-Caspi^1,*,#
Congenital Heart Disease, Vol.15, No.4, pp. 239-249, 2020, DOI:10.32604/CHD.2020.011576 - 07 September 2020
Abstract Objective: To test the hypothesis that circulating miRNAs-146a, -146b, -155, and -21 reflect the inflammatory state of children following heart surgery, and that they may, therefore, correlate with postoperative parameters. We aimed to quantify miRNAs in blood samples from pediatric patients before and 6, 12, and 24 hours after surgery and to evaluate correlations between the miRNA levels and the postoperative course. Setting: PICU. Patients: Forty-two pediatric patients with CHD who underwent cardiac surgery at Safra Children’s Hospital between 2012–2016. Interventions: none. Outcome Measures: The primary outcomes were the postoperative cardiac complications and the secondary outcomes were the More >

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1607

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ARTICLE

Evan Pagano¹, Benjamin Frank¹, James Jaggers², Mark Twite³, Tracy T. Urban⁴, Jelena Klawitter^2,#, Jesse Davidson^1,#,*
Congenital Heart Disease, Vol.15, No.4, pp. 251-265, 2020, DOI:10.32604/CHD.2020.012219 - 07 September 2020
Abstract Objectives: (1) To measure the global shift in the metabolome in hypoxemic versus non-hypoxemic infants with congenital heart disease; (2) To identify metabolites and metabolic pathways that are altered in hypoxemia. Study Design: Analysis of serum samples obtained prior to cardiopulmonary bypass from 82 infants ≤120 days old with congenital heart disease requiring surgery at Children’s Hospital Colorado. Infants were divided into groups based on preoperative oxygen saturations: non-hypoxemic (>92%), mild hypoxemia (85–92%), and severe hypoxemia (<85%). Tandem mass spectrometry was used to analyze 165 targeted metabolites. Partial least squares discriminant analysis and t-tests were used to… More >

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1628

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CASE REPORT

Alejandro R. Peirone^1,*, Alejandro E. Contreras², Carolina Carrizo², Mailén Konicoff², Raúl O. Cayre³
Congenital Heart Disease, Vol.15, No.4, pp. 267-274, 2020, DOI:10.32604/CHD.2020.013199 - 07 September 2020
Abstract A 43-year-old woman with a past medical history of aortic coarctation surgically repaired at the age of 3 years using an end-to-end anastomosis, presented with 2 years complain of increasing dyspnea and fatigue with exercise associated to frequent palpitations. During extensive work-up, she was found to have a partial anomalous pulmonary venous connection (PAPVC) with “dual drainage” represented by a communication between the right pulmonary veins draining into the left atrium and the innominate vein via an anomalous vein due to a persistence of early connections between the sinus of the right pulmonary veins and More >

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