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Objective: Exercise capacity is impaired in Fontan palliated patients. The change in daily activity behaviour with an increase in sedentary lifestyle results in low physical activity levels. This might have a greater impact on patients with chronic heart disease in contrast to healthy controls. For a better understanding, we compared data from cardiopulmonary exercise testing (CPET) with heart rates and physical activity in daily life.
Methods: 21 Fontan patients and 20 age, sex and BMI matched controls underwent CPET and 5 days of daily life activity tracking with a triaxial accelerometer (wGT3x-BT, Actigraph) including heart rate monitoring with an optical heart rate sensor.
Results: 27% of our Fontan teenagers and 71% of the Fontan adults reached the specific WHO recommendations for moderate to vigorous physical activity (MVPA) during everyday life (EDL), without differences to controls. There was a strong correlation between MVPA and [Formula: see text] for all Fontan patients (p = 0.0035, Pearson r = 0.788). Daily MVPA correlated to peak oxygen uptake and lactate production. Up to workloads of 2 W/kg and in daily life heart rates in Fontan patients were similar to controls.
Conclusions: Daily MVPA is alarmingly low without any differences between Fontan patients and controls. Heart rate behaviour was similar and does not seem to be a limiting factor for physical activity in daily life. Higher intensity activities should be implemented regularly in EDL for Fontan patients. Proof is needed as to whether sports in moderate or possibly even in vigorous activity (e.g. high-intensity interval training) improve exercise capacity the most.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6992185 | PMC |
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0228255 | PLOS |
Int J Cardiol Congenit Heart Dis
March 2024
Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Introduction: Each year the number of combined heart-liver transplants (HLT) increases, with two distinct patient populations proceeding down this pathway. The first are patients with congenital heart disease (CHD), most commonly single ventricle patients palliated with Fontan. The second group are those with long standing congestive hepatopathy, amyloidosis, hemochromatosis, or alcohol induced myopathies and liver disease.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
December 2024
Paediatric Diabetes and Endocrine Service, Starship Child Health, Te Toka Tumai Auckland Te Whatu Ora, Auckland, New Zealand.
Objective: Short stature, central hypothyroidism and infertility are common in those with a Fontan circulation. Given that the Fontan circulation often results in hepatic portal venous congestion, we hypothesize that the hypothalamic-pituitary portal circulation is also affected, contributing to subsequent hypothalamic-pituitary axis dysfunction.
Methods: MRI data from the Australian and New Zealand Fontan Registry (86 cases) was compared to 86 age- and sex-matched normal published controls.
Int J Cardiol Congenit Heart Dis
June 2024
University of Sydney, Sydney, Australia.
Introduction: Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people.
Methods: We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan-surgery.
Int J Cardiol Congenit Heart Dis
June 2024
Department of Radiology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea.
Background: Extracardiac conduit Fontan procedure (ECFP) employing a Gore-Tex conduit has been widely used for patients with single ventricle physiology; however, the long-term status of the conduit is unknown. We investigated the changes in a Gore-Tex conduit after ECFP and the factors associated with its narrowing.
Methods: We conducted a retrospective analysis of 86 patients who underwent ECFP between January 1995 and December 2008 and had cardiac computed tomography (CT) during the follow-up period.
Int J Cardiol Congenit Heart Dis
September 2024
Cardiology Department, La Paz University Hospital, Paseo de la Castellana, 261, 28046 Madrid, Spain.
Background: Obstructive complications in the Fontan circulation pose significant risks to affected patients. Traditional management strategies may be insufficient, necessitating innovative treatment approaches. This case report discusses the use of the FlowTriever Retrieval System for percutaneous aspiration thrombectomy in a patient with a history of Fontan procedure who developed conduit obstruction due to thrombus formation.
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