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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]