Honghao Fu^#, Zhangwei Wang^#, Shoujun Li^*

Congenital Heart Disease, Vol.20, No.3, pp. 287-303, 2025, DOI:10.32604/chd.2025.067619 - 11 July 2025

Abstract Congenital heart disease (CHD) stands as the most common cardiovascular disorder among children, exerting a profound impact on the growth, development, and quality of life of the affected pediatric population. The modified Fontan procedure, the total cavopulmonary connection (TCPC), has become a pivotal palliative or definitive surgical method for treating complex CHD cases, including single ventricle and tricuspid valve atresia. Through staged surgical processes, this technique directly diverts vena cava blood into the pulmonary artery, thus improving the patient’s oxygenation status. Despite the initial success of the Fontan circulation in providing a means for survival More > Graphic Abstract

Yuen Lo Yau¹, Matthew S. Purlee², Lindsey M. Brinkley², Dipankar Gupta¹, David M. Saulino³, Dalia Lopez-Colon¹, John-Anthony Coppola¹, Dhanashree Rajderkar⁴, Himesh Vyas^1,*

Congenital Heart Disease, Vol.20, No.2, pp. 265-272, 2025, DOI:10.32604/chd.2025.065661 - 30 April 2025

Abstract Background: Patients with Fontan physiology are predisposed to congestive hepatopathy, progressive liver fibrosis, and end-stage liver disease. Ultrasound-based shear wave elastography (SWE) is a non-invasive tool to diagnose and monitor liver fibrosis. We sought to determine whether the degree of hemodynamic derangement prior to and after the Fontan operation is associated with increased liver stiffness measured by SWE. Methods: A single-center retrospective study of patients with Fontan circulation who underwent ultrasound elastography between 2008 and 2024 was conducted. Liver stiffness was measured by SWE and reported as velocity in m/s. Hemodynamic assessment with cardiac catheterization and… More >

Vatche Bahudian, John Valdovinos^*

Congenital Heart Disease, Vol.20, No.1, pp. 115-127, 2025, DOI:10.32604/chd.2025.063991 - 18 March 2025

Abstract Background: The population of Fontan patients, patients born with a single functioning ventricle, is growing. There is a growing need to develop algorithms for this population that can predict health outcomes. Artificial intelligence models predicting short-term and long-term health outcomes for patients with the Fontan circulation are needed. Generative adversarial networks (GANs) provide a solution for generating realistic and useful synthetic data that can be used to train such models. Methods: Despite their promise, GANs have not been widely adopted in the congenital heart disease research community due, in some part, to a lack of knowledge… More >

William H. Marshall V^1,2,*, Lydia K. Wright²

Congenital Heart Disease, Vol.19, No.4, pp. 419-433, 2024, DOI:10.32604/chd.2024.056608 - 31 October 2024

Abstract Heart failure (HF) is common in patients with congenital heart disease (CHD) and there are limited medical therapies. Sodium-glucose cotransporter 2 inhibitors (SGLT2i) are a proven medical therapy in patients with acquired HF, though data are limited in patients with CHD. The aim of this review is to summarize the current evidence for use of SGLT2i in patients with CHD and identify future directions for study. In available publications, SGLT2i in patients with CHD seem to be well tolerated, with similar side effect profile to patients with acquired HF. Improvement in functional capacity and natriuretic More >

Ryan G. McQueen¹, Nikki M. Singh², Ronald K. Woods^3,*

Congenital Heart Disease, Vol.19, No.3, pp. 257-266, 2024, DOI:10.32604/chd.2024.052108 - 26 July 2024

Abstract Objective: The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood (FFN) to those without a prior Norwood (FF). Methods: A single-institution retrospective review of all patients with Fontan failure who underwent cardiac transplantation from 2003–2021 was completed—22 underwent prior Norwood (FFN) and 11 did not (FF). Descriptive and inferential statistics were calculated for operative course and patient outcomes. Results: The operative course of the FFN cohort appeared to be more complex (not statistically significant, but clinically relevant)—this group… More >

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 - 19 January 2024

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… More > Graphic Abstract

Charis Tan^1,2 , Diana Zannino³, Carley Clendenning³, Sophie Offen⁴, Thomas L. Gentles⁵, Julian Ayer⁶, David Tanous⁷, Vishva Wijesekera⁸, Leeanne Grigg⁹, David Celermajer^2,4,10, Mark McGuire^2,4 , Yves d’Udekem^3,11,12, Rachael Cordina^2,4,10,*

Congenital Heart Disease, Vol.18, No.5, pp. 507-523, 2023, DOI:10.32604/chd.2023.028829 - 10 November 2023

Abstract Objective: Sudden cardiac death (SCD) and malignant ventricular arrhythmia (VA) are increasingly recognized as important issues for people living with a Fontan circulation, but data are lacking. We sought to characterize the cohort who had sudden cardiac death, most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: Of 1611 patients, 20 (1.2%) had VA;… More >

Nerejda Shehu^1,*, Christian Meierhofer¹, Anja Hennemuth^2,3, Markus Hüllebrand^2,3, Pavlo Yevtushenko³, Peter Ewert¹, Stefan Martinoff⁴, Heiko Stern¹

Congenital Heart Disease, Vol.18, No.4, pp. 461-474, 2023, DOI:10.32604/chd.2023.031075 - 15 September 2023

Abstract Background: Pressure measurement in total cavopulmonary connection (TCPC) patients is a domain of cardiac catheterization. 4D velocity encoded cardiovascular magnetic resonance (4D–flow MRI) offers an alternative for assessment of even minor pressure differences. The scope of this study was to measure even minor pressure differences in the anastomosis of TCPC patients, who are clinically uncompromised. Methods: Twenty-four patients (median 15 years [8;34]) with TCPC were studied prospectively by 4D-flow MRI. Pressure differences between superior vena cava (SVC) and extracardiac conduit (C) to both right pulmonary artery (RPA) and left pulmonary artery (LPA) were assessed. Small… More > Graphic Abstract

Yuki Kawasaki^1,*, Takeshi Sasaki¹, Daisuke Kobayashi²

Congenital Heart Disease, Vol.18, No.4, pp. 431-445, 2023, DOI:10.32604/chd.2023.030910 - 15 September 2023

Abstract Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized that hemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinical features and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients who underwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI  ≥ 3, SVR index (SVRI) ≥ 20; B, CI < 3, SVRI ≥ 20; C, CI ≥ 3, SVRI < 20; and D, CI < 3, SVRI < 20. The primary outcome was freedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant, and death. Clinical and… 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 - 09 January 2023

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… More > Graphic Abstract