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  • Open Access

    ARTICLE

    The Norwood with Blalock-Taussig-Thomas Shunt—Worth a Second Look?

    Manasa Gadiraju1,*, Dhaval Chauhan2, Alexandra Schray3, James E. O’Brien Jr.1,4, Edo Kwaku Setsoafia Bedzra1,4

    Congenital Heart Disease, Vol.19, No.5, pp. 435-443, 2024, DOI:10.32604/chd.2025.059705 - 31 December 2024

    Abstract Background: Shunt repair for the Norwood procedure with either the right ventricle to pulmonary artery shunt (RVPAS) or the modified Blalock-Taussig-Thomas Shunt (BTTS) varies by institution and surgeon preference. Shunt choice has been informed by landmark trials including the Single Ventricle Reconstruction trial and modern outcomes data may engender future complementary studies. Methods: We conducted a retrospective analysis of all patients who underwent the Norwood procedure from 2014–2022 at a single center to compare outcomes by shunt type. The primary outcome measure was freedom from death or transplant. Secondary outcome measures included hospital length of… More >

  • Open Access

    ARTICLE

    Results of Fontan in Patients with Apicocaval Juxtaposition or/and Separated Hepatic Venous Drainage

    Ju Wang, Shuo Dong, Jun Yan*

    Congenital Heart Disease, Vol.16, No.5, pp. 477-485, 2021, DOI:10.32604/CHD.2021.015016 - 03 June 2021

    Abstract Objective: Modifications of the Fontan operation, which are also known as total cavopulmonary connection (TCPC), are widely applied for patients with functionally univentricular hearts (FUH). Herein, we summed up the different surgical pathways and clinical outcomes in FUH patients with apicocaval juxtaposition (ACJ) or/and separated hepatic venous (SHV) drainage. Methods: Between January 2009 and December 2019, 123 patients who undergone TCPC in our institute were included in this retrospective study. We have included 70 patients with ACJ (Group 1) and 53 patients with SHV (Group 2). Moreover, Group 2 included 17 cases combing with ACJ (32.1%). In… More >

  • Open Access

    ARTICLE

    Progressive loss of bone mass in children with Fontan circulation

    Simone Goa Diab1, Kristin Godang2, Lil‐Sofie Ording Müller3, Runar Almaas4, Charlotte de Lange3, Leif Brunvand1, Kari Margrethe Hansen1, Anne Grethe Myhre5, Gaute Døhlen1, Erik Thaulow1,6, Jens Bollerslev2,6, Thomas Möller1

    Congenital Heart Disease, Vol.14, No.6, pp. 996-1004, 2019, DOI:10.1111/chd.12848

    Abstract Objective: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables.
    Methods: A cross‐sectional sample of 64 patients was examined with dual‐energy X‐ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4‐9 years old (n = 22), B: 10‐15 years old (n =… More >

  • Open Access

    ARTICLE

    A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

    Alessia Callegari1,2,4, Rhoia Neidenbach2, Ornella Milanesi1, Biagio Castaldi1, Martin Christmann4, Masamichi Ono3, Jan Müller2, Peter Ewert2, Alfred Hager2

    Congenital Heart Disease, Vol.14, No.2, pp. 147-155, 2019, DOI:10.1111/chd.12694

    Abstract Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
    Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
    Results: Overall, mean forced… More >

  • Open Access

    ARTICLE

    Optimum age for performing Fontan operation in patients with univentricular heart

    Emmanuel Akintoye1, Gruschen R. Veldtman2, William R. Miranda3, Heidi M. Connolly3, Alexander C. Egbe3

    Congenital Heart Disease, Vol.14, No.2, pp. 138-139, 2019, DOI:10.1111/chd.12690

    Abstract The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in‐hospital mortality, procedure‐related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years. More >

  • Open Access

    REVIEW

    Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation

    Laura S. Kverneland1,2, Peter Kramer2, Stanislav Ovroutski2

    Congenital Heart Disease, Vol.13, No.2, pp. 181-193, 2018, DOI:10.1111/chd.12570

    Abstract Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients’ characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published… More >

  • Open Access

    ARTICLE

    Comparative Evaluation of Flow Quantification across the Atrioventricular Valve in Patients with Functional Univentricular Heart after Fontan’s Surgery and Healthy Controls: Measurement by 4D Flow Magnetic Resonance Imaging and Streamline Visualization

    Hoi Lam She*, Arno A.W. Roest, Emmeline E. Calkoen, Pieter J. van den Boogaard, Rob J. van der Geest, Mark G. Hazekamp§, Albert de Roos, Jos J.M. Westenberg

    Congenital Heart Disease, Vol.12, No.1, pp. 40-48, 2017

    Abstract Purpose. To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan’s operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach.
    Method. Seven patients with functional univentricular heart after Fontan’s operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured. Transatrioventricular flow quantification was assessed using conventional 2D multiplanar reformation (MPR) and 4D MPR tracking the annulus and perpendicular to the streamline inflow at… More >

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