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  • Open Access


    Analysis of Risk Factors for Early Mortality in Surgical Shunt Palliation: Time for a Change?

    François-Xavier Van Vyve1, Karlien Carbonez2, Jelena Hubrechts2, Geoffroy de Beco1, Jean E. Rubay1, Mona Momeni3, Thierry Detaille4, Alain J. Poncelet1,*

    Congenital Heart Disease, Vol.18, No.5, pp. 539-550, 2023, DOI:10.32604/chd.2023.042344

    Abstract Objectives: Over the last decade, neonatal repair has been advocated for many congenital heart diseases. However, specific subgroups of complex congenital heart disease still require temporary palliation for which both surgical and endovascular techniques are currently available. We reviewed our institutional experience with shunt palliation with an emphasis on risk factors for early mortality. Methods: This is a single-center retrospective study on 175 patients undergoing surgery for central shunt or modified Blalock-Taussig shunt. All data were extracted from a prospectively collected computerized database. We identified risk factors for early mortality by uni- and multi-variable analysis. All data were censored at… More >

  • Open Access


    Analysis of Pulmonary Arteries Growth after Initial Shunt Palliation in Neonates and Infants

    François-Xavier Van Vyve1,#, Karlien Carbonez2,#, Geoffroy de Beco1, Stéphane Moniotte2, Jean E. Rubay1, Mona Momeni3, Laurent Houtekie4, Alain J. Poncelet1,*

    Congenital Heart Disease, Vol.18, No.5, pp. 525-537, 2023, DOI:10.32604/chd.2023.042341

    Abstract Objective: Despite increasing enthusiasm for neonatal repair, patients with ductal-dependent circulation (pulmonary/systemic) or restrictive pulmonary blood flow still require initial palliation. Ductal stenting has emerged as an endovascular approach whereas modified-Blalock-Taussig and central shunt remain surgical references. In this study, we analyzed the relationship between pulmonary artery growth, sites of shunt connection, or antegrade pulmonary blood flow in surgically placed shunts. The need for secondary catheter-based interventions or pulmonary arterioplasty was also investigated. Methods: A retrospective single-center study analyzing 175 patients undergoing surgery for a central or modified-Blalock-Taussig shunt. Outcome growth variables were right pulmonary artery/left pulmonary artery diameters/Z scores,… More > Graphic Abstract

    Analysis of Pulmonary Arteries Growth after Initial Shunt Palliation in Neonates and Infants

  • Open Access


    Transcatheter Closure of a Right Pulmonary Artery to Left Atrium Fistula Using a Ventricular Septal Defect Occluder

    Diqi Zhu1, Xiaohong Gu2, Jie Shen1,*, Fen Li1,*

    Congenital Heart Disease, Vol.16, No.2, pp. 147-150, 2021, DOI:10.32604/CHD.2021.014360

    Abstract Background: Communication between the right pulmonary artery (RPA) and left atrium (LA) is a rare cause of central cyanosis in pediatric patients. Case presentation: We describe a 3-year-old female patient with an oxygen saturation of 70% at admission. The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization. The angiography of pulmonary artery revealed a 7.4 mm × 7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion. Conclusion: The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect. It… More >

  • Open Access


    Alterations in Metabolites Associated with Hypoxemia in Neonates and Infants with Congenital Heart Disease

    Evan Pagano1, Benjamin Frank1, James Jaggers2, Mark Twite3, Tracy T. Urban4, Jelena Klawitter2,#, Jesse Davidson1,#,*

    Congenital Heart Disease, Vol.15, No.4, pp. 251-265, 2020, DOI:10.32604/CHD.2020.012219

    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 determine differences among… More >

  • Open Access


    Pheochromocytoma and paraganglioma in Fontan patients: Common more than expected

    Mi Kyoung Song1, Gi Beom Kim1, Eun Jung Bae1, Young Ah Lee1, Hyun-Young Kim2, Seung-Kee Min3, Jung Hee Kim4, Jae-Kyung Won5

    Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625

    Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
    Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s Hospital.
    Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5%… More >

  • Open Access


    Subclinical hypothyroidism: A common finding in adult patients with cyanotic congenital heart disease

    Peter Bak1, Cristel S. Hjortshøj2, Peter Gæde3, Lars Idorn4, Lars Søndergaard2, Annette S. Jensen2

    Congenital Heart Disease, Vol.13, No.2, pp. 263-270, 2018, DOI:10.1111/chd.12565

    Abstract Objective: Cyanotic congenital heart disease is a systemic disease, with effects on multiple organ systems. A high prevalence of subclinical hypothyroidism (SCH) has been reported in a small cohort of cyanotic congenital heart disease patients. Subclinical hypothyroidism has been associated with various adverse cardiovascular effects, as well as an increased risk of progression to overt hypothyroidism. The aim of this study was to examine the prevalence of SCH in cyanotic congenital heart disease patients, consider possible etiologies, and evaluate thyroid function over time.
    Methods: First, 90 clinically stable cyanotic congenital heart disease patients were examined with blood samples (thyroid-stimulating hormone,… More >

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