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Fontan patients' exercise capacity and associated risks of morbidity and mortality necessitate a comprehensive study. We aim to explore maximal and submaximal exercise capacity, assessing the impact of cardiovascular and peripheral factors, including atrial strain, aortic stiffness, and muscle-related factors. Fontan patients and matched controls were examined. Clinical details were recorded, and atrial strain and aortic stiffness were measured using echocardiography. Hand grip and knee extensor strength were assessed, and muscle oxygenation was recorded. Cardiopulmonary exercise test determined exercise capacity, with peak oxygen uptake/kg (VO/kg) and oxygen uptake efficiency slope/kg (OUES/kg) as markers for maximal and submaximal capacity, respectively. Thirty-one Fontan patients (median age = 18, range = 7-31 years) and 30 controls (median age = 18, range = 7-32 years) were studied. No significant differences in body composition and muscle strength were observed between Fontan patients and controls (p > 0.05). Fontan patients exhibited lower peak VO/kg and OUES/kg (p < 0.001 for both), along with decreased atrial strain and increased aortic stiffness compared to controls (p < 0.001). Aortic pulse wave velocity (PWV), left atrial strain contractile phase (LASct), and knee extensor strength independently predicted peak VO/kg (R = 0.514) and OUES/kg (R = 0.486) in Fontan patients; age was a predictor for peak VO/kg. Atrial contractile strain, aortic stiffness, and knee extensor muscle strength are predictors of submaximal exercise capacity; furthermore, age, along with these variables, serves as predictors of maximal exercise capacity in Fontan patients. Evaluating hemodynamic, vascular, and muscular parameters, in conjunction with assessing both maximal and submaximal exercise capacities, is crucial for optimizing disease management in Fontan patients. Clinicaltrials.gov registration: NCT05011565.
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http://dx.doi.org/10.1007/s00246-024-03687-3 | DOI Listing |
Cardiol Young
September 2025
Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
The Occlutech Atrial Flow Regulator is a self-expandable double-disc nitinol device with a central fenestration designed to create a calibrated communication across the interatrial septum. It has been used in adult patients with heart failure and pulmonary hypertension. Its use in the paediatric population or adults with CHD has been published in several case reports and case series.
View Article and Find Full Text PDFPatients with cardiovascular compromise are likely to develop hypotension upon receiving even small doses of sedatives. On the other hand, patients with severe dental phobias or with intellectual disability who have a severe gag reflex often require deeper levels of anesthesia. Thus, achieving an optimal level of anesthesia can be difficult in patients with cardiovascular compromise because of the relatively narrow range of sedative dosing capable of providing sufficient sedation to prevent the gag reflex without compromising hemodynamics.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
September 2025
Texas Center for Pediatric and Congenital Heart Disease, The University of Texas at Austin Dell Medical School, Austin, TX, USA.
Pericardial effusion (PCE) represents a significant postoperative complication following congenital heart surgery (CHS), contributing to more complex postoperative care and heightened morbidity. In this study, we aim to elucidate the risk factors contributing to PCE development post-CHS through analysis of data from a nationwide, multi-institutional database. Review of the Pediatric Health Information System Database from January 1, 2004, to December 30, 2023.
View Article and Find Full Text PDFJTCVS Open
August 2025
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, Washington, DC.
Objectives: We sought to review the outcomes of patients with Ebstein anomaly (EA) after the Fontan operation.
Methods: Patients with EA were identified from a large binational registry about the Fontan operation. Data were collected from hospital records, registry data, and clinical correspondence.
JTCVS Open
August 2025
Division of Cardiothoracic Surgery, Department of Surgery, University of Southern California, Los Angeles, Calif.
Objective: Patients with heterotaxy-associated congenital heart disease often require multiple operations, which may have a cumulative effect on their outcomes. This study aimed to define the cardiac surgical course in a large cohort and identify longitudinal risk factors for death/transplant.
Methods: All patients with heterotaxy-associated congenital heart disease who underwent cardiac surgery at one institution from 2005 to 2022 were retrospectively reviewed.