Elisa Listo^1,#, Nicola Martini^2,#, Stefano Salvadori³, Elisa Valenti³, Nicola Stagnaro¹, Gianluca Trocchio⁴, Chiara Marrone⁵, Alberto Clemente⁶, Francesca Raimondi^7,*, Pierluigi Festa⁵, Lamia Ait Ali^5,8,*

Congenital Heart Disease, Vol.18, No.6, pp. 627-638, 2023, DOI:10.32604/chd.2023.030312

Abstract Background: The assessment of Fontan circuit’s flow is traditionally evaluated by multiple through-plane phase-contrast MRI acquisitions (2D flow), while recently, a single volumetric 4D-flow MRI acquisition is emerging as a comprehensive tool for the hemodynamic evaluation in congenital heart diseases. Purpose: To compare 2D and 4D-flow MRI measurements in patients after Fontan palliation and to evaluate parameters affecting potential disagreement. Methods: 39 patients after Fontan palliation (23 males, age 22 ± 11 years) who underwent cardiac MRI with 2D and 4D-flow MRI acquisition were included in the study. In all patients, blood flow quantification in the Fontan circuit and aorta… More > Graphic Abstract

Jinlong Liu^1,2,#, Jing Shi^3,#, Weiru Luo¹, Zhirong Tong^1,2, Lefei Yang³, Peixuan Sun³, Tianyi Li³, Jun Du^3,*, Qian Wang^3,*

Congenital Heart Disease, Vol.18, No.1, pp. 41-55, 2023, DOI:10.32604/chd.2023.025005

Abstract Background: The assessment of renal function is important to the prognosis of patients needing Fontan palliation due to the reconstructed compromised circulation. To know the relationship between the kidney perfusion and hemodynamic characteristics during surgical design could reduce the risk of acute kidney injury (AKI) and the postoperative complications. However, the issue is still unsolved because the current clinical evaluation methods are unable to predict the hemodynamic changes in renal artery (RA). Methods: We reconstructed a three-dimensional (3D) vascular model of a patient requiring Fontan palliation. The technique of computational fluid dynamics (CFD) was utilized to explore the changes of… More > Graphic Abstract

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

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 creatinine-based Full Age… More >

Emily M. Engelhardt¹, Andrew T. Trout^2,3, Rachel M. Sheridan⁴, Gruschen R. Veldtman⁵, Jonathan R. Dillman^2,3

Congenital Heart Disease, Vol.14, No.3, pp. 380-388, 2019, DOI:10.1111/chd.12730

Abstract Purpose: Patients who have undergone Fontan palliation of single ventricle physiol‐ ogy congenital heart disease are prone to developing focal liver lesions. In our experi‐ ence, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy‐ proven liver lesions in patients post–Fontan palliation of single ventricle physiology cared for at our institution.
Methods: We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records were reviewed for… More >

Alessia Callegari^1,2,4, 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 expiratory volume… More >

Neil C. Zaki^1,2, Michael S. Kelleman¹, W. James Parks^1,3, Timothy C. Slesnick^1,3, Michael E. McConnell^1,3, Matthew E. Oster^1,3

Congenital Heart Disease, Vol.14, No.2, pp. 140-146, 2019, DOI:10.1111/chd.12692

Abstract Objective: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years postFontan operation. The purpose of this study was to determine the impact of this standardized practice of cMRI screening on the clinical management of a Fontan population.
… More >