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Background: The ongoing COVID-19 pandemic has infected more than 500 million people worldwide. Several measures have been taken to reduce the spread of the virus and the saturation of intensive care units: among them, a lockdown (LD) was declared in Italy on 9 March 2020. As a result, gyms, public parks, sports fields, outdoor play areas, schools, and multiple commercial activities have been closed. The consequences of physical inactivity can be dramatic in adult patients with congenital heart disease (ACHD), in which the benefit of regular exercise is well known. In this study, we investigated the effects of reduced physical activity during the COVID-19 pandemic on ACHD's exercise capacity. Materials and Methods: Patients who performed exercise or cardiopulmonary exercise tests from October 2019 to February 2020 and one year after lockdown with the same protocol were retrospectively enrolled in our database. Inclusion criteria: ACHD patients aged ≥ 18 years old under regular follow-up. Exclusion criteria: significant clinical and/or therapeutic changes between the two tests; significant illness occurred between the two tests, including COVID-19 infection; interruption of one of the tests for reasons other than muscle exhaustion. Results: Thirty-eight patients (55.6% males) met the inclusion criteria. Before the lockdown, 17 patients (group A) were engaged in regular physical activity (RPA), and 20 patients (group B) had a sedentary lifestyle. After LD, in group A, (a) the weekly amount of physical activity reduced with statistical significance from 115 ± 46 min/week to 91 ± 64 min/week (−21%, p = 0.03); (b) the BMI did not change; (c) the duration of exercise test and VO2 max at cardiopulmonary exercise test showed a significant reduction after the LD. In group B, BMI and exercise parameters did not show any difference. Conclusions: The COVID-19 pandemic dramatically changed the habits of ACHD patients, significantly reducing their possibility to exercise. Our data analyzed in this extraordinary situation again demonstrated that physical inactivity in ACHD worsens functional capacity, as highlighted by VO2 max. Regular exercise should be encouraged in ACHD patients to preserve functional capacity. [ABSTRACT FROM AUTHOR]

Simple Summary: Fontan-associated liver disease (FALD) is a late sequela of single ventricle palliation. The long-term consequences of Fontan's physiology have a spectrum of outcomes resulting in chronic hepatic venous congestion, liver cirrhosis, hyperplastic nodules, and liver neoplasms that are both benign, such as hepatic adenoma (HA), and malignant, including hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC). To date, genetic and molecular studies of HCC/CC contextual to FALD are lacking. We report the molecular profile of the heterogeneous group of tumors observed in the FALDs (HA, HCC, and CC) we studied with the aim of comparing any differences with the same tumors without FALD and identifying possible recurrent variables, such as molecular alterations, that could be predictive of clinical outcome. Purpose: Univentricular heart is corrected with the Fontan procedure (FP). In the long term, so-called Fontan-associated liver diseases (FALDs) can develop. The aim of this study is to analyze the molecular profile of FALDs. Methods: FALDs between January 1990 and December 2022 were reviewed for histology and immunohistochemistry, laboratory data, and images. Targeted next generation sequencing (NGS), performed on the DNA and RNA of both neoplastic and non-lesional liver tissue, was applied. Results: A total of 31/208 nodules > 1 cm in diameter were identified on imaging, but a liver biopsy was available for five patient demonstrating the following: one hepatocellular adenoma (HA), two hepatocellular carcinomas (HCCs), one fibrolamellar carcinoma (FLC), and one intrahepatic cholangiocarcinoma (ICC). Molecular analysis showed a copy number alteration involving FGFR3 in three cases (two HCCs and one ICC) as well as one HCC with a hotspot mutation on the CTNNB1 and NRAS genes. Tumor mutational burden ranged from low to intermediate. A variant of uncertain significance in GNAS was present in two HCCs and in one ICC. The same molecular profile was observed in a non-lesional liver. A DNAJB1-PRKACA fusion was detected only in one FLC. Conclusions: Neoplastic FALDs show some unusual molecular profiles compared with non-Fontan ones. The presence of the same alterations in non-lesional cardiac cirrhosis could contribute to the development of FALD. [ABSTRACT FROM AUTHOR]

Singleton pregnancy in Fontan patients is burdened by a significant maternal cardiovascular and obstetric risk. The cardiac workload in a twin pregnancy is greater and could place Fontan-palliated patients at an increased risk of complications. We report a case of a woman with Fontan circulation and homozygous MTHFR mutation who had a twin pregnancy., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Over recent years, thanks to remarkable advances in pediatric cardiology, cardiac surgery and catheter interventions, survival of children with congenital heart disease has significantly increased with the majority of patients surviving into adulthood. Therefore, the prevalence of adult patients with congenital heart disease has dramatically increased, as well as the need for specific and dedicated programs. Acute heart failure, infective endocarditis and arrhythmias represent the most common causes of visit in the emergency department in this population. Our task force aimed at guiding physicians taking care of this peculiar cohort of patients in the emergency department.

Introduction: Advances in 3D electroanatomic-mapping technologies have resulted in a safe and effective profile of radiofrequency (RF) catheter ablation. The aim of this study was to evaluate a different catheter ablation approach in patients with left-sided accessory pathways (APs)., Methods and Results: From January 2015 to December 2015, 30 patients (median age 11 years, median weight 45 kg) with manifest or concealed left-sided APs underwent RF catheter ablation with a new protocol. All procedures were performed with the CARTO UNIVU™ system, integrating electroanatomic maps with fluoroscopic views. A 7 Fr ablation catheter was inserted into the right femoral vein and advanced into the right atrium. Geometrical reconstruction and activation map of the right atrium, tricuspid annulus, and coronary sinus were acquired. The ablation catheter was then inserted into the left femoral artery and advanced through the aorta and aortic valve, creating an activation map of the mitral annulus. Catheter ablation was targeted to the site of the earliest activation. No complications occurred. The median procedure and fluoroscopy times were 130 min and 6 s, with a median fluoroscopy dose 0.5 mGy. An average of two catheters was used. Long-term success rate was 97% (29/30) at a median follow-up of 9.6 months. This approach reduced fluoroscopy time, dose and number of catheters used compared with manifest or concealed left-sided AP ablation using CARTO 3™ (P < 0.05)., Conclusions: This new ablation protocol seems to be promising in reducing fluoroscopy exposure and number of catheters used during left-sided AP ablation in children., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions please email: journals.permissions@oup.com.)

Aims: Children and adolescents with ventricular pre-excitation (VPE) are at increased risk for sudden cardiac death (SCD). Although antiarrhythmic therapy and catheter ablation are well established temporary or definitive treatments for patients with Wolff-Parkinson-White (WPW) syndrome, the optimal management of children with asymptomatic VPE remains to be clearly defined. On the basis of the most recent guidelines and recommendations, the aim of this study was to determine the electrophysiological characteristics of young patients with VPE and WPW syndrome to assess and compare their potential risk of SCD., Methods and Results: We retrospectively investigated 124 consecutive young patients with VPE (51 with WPW syndrome and 73 asymptomatic) who underwent transoesophageal electrophysiological study. At baseline, atrioventricular reentrant tachycardia (AVRT) was induced in 13 WPW vs. 10 asymptomatic patients (25.5 vs. 13.7%, P = NS). Atrial fibrillation (AF) was induced in 13 WPW vs. 15 asymptomatic patients (25.5 vs. 20.5%, P = NS). A shortest pre-excited R-R interval (SPERRI) ≤250 ms during AF was found in four WPW vs. six asymptomatic patients (30.8 vs. 40%, P = NS). During isoproterenol infusion or stress testing, AVRT was induced in 31 of 44 WPW vs. 33 of 69 asymptomatic patients (70.4 vs. 47.8%, P = 0.018). Atrial fibrillation was induced in 12 of 44 WPW vs. 21 of 69 asymptomatic patients (27.3 vs. 30.4%, P = NS). A SPERRI ≤ 210 ms was found in 6 of 12 WPW vs. 10 of 21 asymptomatic patients (50 vs. 47.6%, P = NS). No statistically significant correlation was observed between accessory pathway location and symptoms, AVRT/AF inducibility, or mean APERP/SPERRI values., Conclusion: Children and adolescents with WPW syndrome have a higher rate of AVRT inducibility than asymptomatic patients. However, no differences between the two groups were found in atrial vulnerability and parameters related to the risk of SCD., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

Objective: Atherosclerosis is characterized by endothelial inflammation and dysfunction. Adipose tissue has increasingly been recognized as an active endocrine organ secreting so-called adipokines. Among these, resistin--recently described, but not yet extensively studied--has been defined as a novel inflammatory marker in atherosclerosis. The pathophysiology underlying this interplay, however, remains to be fully characterized. The aim of the study is to determine whether resistin might affect prothrombotic characteristics of human coronary artery endothelial cells (HCAECs)., Methods and Results: Incubation of HCAECs with resistin caused upregulation of tissue factor (TF) expression as demonstrated by FACS analysis. Moreover, TF activity was induced in a dose-dependent manner, as shown by real-time PCR and colorimetric assay. Resistin-induced TF expression was mediated by oxygen free radicals through the activation of the transcription factor nuclear factor-κB (NF-κB), as demonstrated by electrophoretic mobility shift assay and by suppression of TF expression by superoxide dismutase, catalase, and the NF-κB inhibitors PDTC and BAY 11-7082., Conclusions: These data confirm the hypothesis that resistin may contribute to atherothrombosis, exerting direct effects on HCAECs by promoting TF expression; thus, it represents an effector molecule able to induce a prothrombotic phenotype in cells present in the vessel wall., (Copyright © 2010 S. Karger AG, Basel.)