In patients after Fontan completion exercise capacity is significantly reduced. Although peak oxygen consumption (VO) is a strong prognostic factor in many cardiovascular diseases, it requires the achievement of a maximal effort. Therefore, submaximal exercise parameters such as oxygen uptake efficiency slope (OUES) may be of value. In the present observational study we evaluated the exercise capacity with maximal and submaximal parameters in a group of Fontan patients with an extracardiac conduit and determined their prognostic value. Sixty Fontan patients followed up in the Leiden University Medical Center who have performed an exercise test were included in this retrospective study. Exercise tests were performed at a median age of 11 years. Fontan patients showed on average lower values for all exercise parameters compared to reference values from a healthy dataset as shown by the %predicted values: VO%:mean 66%(95%CI:64 to 74) and OUES%:mean 72%(95%CI:67 to 77). Twenty percent of the patients were not able to achieve an RER>1.0. RER showed a moderate positive correlation with VO but not with OUES. There was a deterioration of VO% and OUES% over time. OUES was significantly lower in patients with cardiac events in the follow up period. Fontan patients have an impaired exercise performance even at young ages and it deteriorates with age. An important percentage of Fontan patients is not able to reach maximal effort so the use of submaximal parameters, like OUES, should be considered as part of the evaluation. Moreover, OUES could have a prognostic value in this group of patients.
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http://dx.doi.org/10.1016/j.amjcard.2021.05.049 | DOI Listing |
Ann Thorac Surg Short Rep
December 2024
Department of Cardiovascular Surgery, Nagano Children's Hospital, Azumino, Japan.
Background: The discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy.
Methods: Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy.
Ann Thorac Surg Short Rep
September 2024
Biostatistics Unit, Department of Data Science, National Center for Child Health and Development, Tokyo, Japan.
Background: The primary treatment for hypoplastic left heart syndrome (HLHS) is the Fontan pathway, which entails performing the Glenn procedure. We hypothesized that the superior vena cava in patients with HLHS was short. As the length of the superior vena cava influences the Glenn procedure, we compared its length between patients with HLHS and those with other congenital heart diseases.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Surgery, The Ohio State University, Columbus, Ohio.
Background: Single-ventricle cardiac defects (SVCDs) are among of the most health care resource-intensive congenital diseases. Although SVCDs are traditionally palliated using the Norwood pathway, in the last 2 decades select programs have used the hybrid strategy, which redistributes the operative and interstage risks. This study sought to characterize resource use for a cohort of patients with hybrid-palliated SVCD.
View Article and Find Full Text PDFCardiovasc Diagn Ther
December 2024
Department of Pediatric Cardiology, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Background And Objective: Cardiovascular magnetic resonance (CMR) is a routine cross-sectional imaging modality in adults with congenital heart disease. Developing CMR techniques and the knowledge that CMR is well suited to assess long-term complications and to provide prognostic information for single ventricle (SV) patients makes CMR the ideal assessment tool for this patient cohort. Nevertheless, many of the techniques have not yet been incorporated into day-to-day practice.
View Article and Find Full Text PDFInt J Cardiovasc Imaging
January 2025
Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany.
T1 relaxation time quantification on parametric maps is routinely used in cardiac imaging and may serve as a non-invasive biomarker for diffuse liver disease. In this study, we aimed to investigate the relationship between liver T1 values and cardiac function in patients with congenital heart disease (CHD) and compared patients with a biventricular circulation (BVC) to those with a Fontan circulation (FC). Magnetic resonance images from patients with CHD, obtained between June and December 2023 on a 1.
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