Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

Hedlund, Eva Rylander; Ljungberg, Henrik; Söderström, Liselott; Lundell, Bo; Sjöberg, Gunnar

doi:10.1017/s1047951118000902

Cited by 22 publications

(39 citation statements)

References 88 publications

(156 reference statements)

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“…About one third of the patients in our cohort exhibited a restrictive breathing pattern at baseline, in line with previous reports on lung function test results in Fontan patients ( 7 , 47 ). The observed restrictive breathing pattern is likely caused by multiple factors, which include diaphragmatic paralysis ( 48 , 49 ) and a reduced thoracic mobility due to repeated thoracotomies with presence of less flexible scar tissue ( 49 ).…”

Section: Discussionsupporting

confidence: 92%

“…Previous results on the impact of different physical training programs on lung function testing in Fontan patients revealed conflicting results. While, similar to our results, Fritz et al failed to show significant improvements in vital capacity, total lung capacity and Tiffeneau-Index (FEV1/VC) after daily inspiratory muscle training over 10 months (50), Hedlund et al demonstrated an increased vital capacity after endurance training (7).…”

Section: Pulmonary Functionsupporting

confidence: 90%

“…In addition, a decreased heart rate reserve, ventricular fibrosis and/or hypertrophy, and an impaired systolic and/or diastolic function all result in a reduced capacity to increase stroke volume and cardiac output during physical activity (2,5). Furthermore, Fontan patients exhibit a restrictive lung function pattern, an impaired pulmonary diffusing capacity, and a reduced maximal oxygen uptake (7), which, in turn, are associated with decreases in health-related quality of life (8)(9)(10)(11) and exercise tolerance (12)(13)(14).…”

Section: Introductionmentioning

confidence: 99%

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Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation—Initial Results From a Prospective Study

Dirks

Krämer

Schleiger

et al. 2022

Front. Cardiovasc. Med.

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BackgroundPatients with congenital heart disease (CHD)—including those after Fontan operation—are encouraged to be physically active.AimTo prospectively determine the effects of an individually adapted, home-based cycle ergometer endurance training in combination with inspiratory muscle training (IMT) in pediatric and adult Fontan patients. We, herein, report the results of the initial 10-months follow-up (phase 1).Methods18 patients (median age 16.5 years; range 10-43 years) completed baseline check-ups, and 4 and 10 months follow-up visits, which each included cardiopulmonary exercise testing (CPET), bodyplethysmography (including measurement of respiratory muscle strength), and a quality of life questionnaire (PedsQL™). The training program consisted of a home-based cycle ergometer endurance training on a “Magbike® AM-5i/3i” (DKN Technology®, Clermont-Ferrand, France) and IMT with a handheld “POWERbreathe® Medic plus” device. Patients performed 90 min of endurance training per week in addition to IMT (30 breaths per day, 6-7 times per week). After the first 4 months, patients underwent additional interval training.ResultsAfter 10 months of training, we observed significant increases in maximum relative workload (W/kg, p = 0.003) and in maximum inspiratory (MIP, p = 0.002) and expiratory (MEP, p = 0.008) pressures. Peak VO2 values did not increase significantly as compared to baseline (p = 0.12) in the entire cohort (n = 18), but reached statistical significance in a subgroup analysis of teenage/adult patients (n = 14; p = 0.03). Patients' subjective quality of life did not show any significant changes after 10 months of training.DiscussionIn Fontan patients, an individually adapted home-based training is safe and associated with improvements in some CPET variables. However, these improvements did not translate into an improved QoL after 10 months. With an unclear, but most likely negative, impact of the COVID-19 pandemic, improvements in QoL may become evident during further follow-up (phase 2 of the study).

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Section: Discussionsupporting

confidence: 92%

Section: Pulmonary Functionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 99%

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Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation—Initial Results From a Prospective Study

Dirks

Krämer

Schleiger

et al. 2022

Front. Cardiovasc. Med.

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“…We have previously published studies of quality of life, 24 physical capacity and effects of endurance training, 25 and lung function 26 in a cohort of young Fontan patients compared with a matched group of healthy children and adolescents. For detailed information about our Fontan cohort, control group, and methods used, we refer to those publications.…”

mentioning

confidence: 99%

Appropriate heart rate during exercise in Fontan patients

2020

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Objective:To evaluate heart rate against workload and oxygen consumption during exercise in Fontan patients.Method:Fontan patients (n = 27) and healthy controls (n = 25) underwent cardiopulmonary exercise testing with linear increase of load. Heart rate and oxygen uptake were measured during tests. Heart rate recovery was recorded for 10 minutes.Results:Heart rate at midpoint (140 ± 14 versus 153 ± 11, p < 0.001) and at maximal effort (171 ± 14 versus 191 ± 10 beats per minute, p < 0.001) of test was lower for patients than controls. Heart rate recovery was similar between groups. Heart rate in relation to workload was higher for patients than controls both at midpoint and maximal effort. Heart rate in relation to oxygen uptake was similar between groups throughout test. Oxygen pulse, an indirect surrogate measure of stroke volume, was reduced at maximal effort in patients compared to controls (6.6 ± 1.1 versus 7.5 ± 1.4 ml·beat−1·m−2, p < 0.05) and increased significantly less from midpoint to maximal effort for patients than controls (p < 0.05).Conclusions:Heart rate is increased in relation to workload in Fontan patients compared with controls. At higher loads, Fontan patients seem to have reduced heart rate and smaller increase in oxygen pulse, which may be explained by inability to further increase stroke volume and cardiac output. Reduced ability to increase or maintain stroke volume at higher heart rates may be an important limiting factor for maximal cardiac output, oxygen uptake, and physical performance.

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“…Patients who had undergone the Fontan procedure are at increased risk of maldistribution of pulmonary blood flow, which in turn is associated with decreased exercise capacity (2,13,28). Impaired lung function, including restrictive lung disease, air trapping, and reduced diffusing capacity are also common and likely compound exercise impairment (20).…”

Section: Discussionmentioning

confidence: 99%

A 4D flow MRI evaluation of the impact of shear-dependent fluid viscosity on in vitro Fontan circulation flow

Cheng

Wee

Pahlevan

et al. 2019

American Journal of Physiology-Heart and Circulatory Physiology

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The Fontan procedure for univentricular heart defects creates a nonphysiologic circulation where systemic venous blood drains directly into the pulmonary arteries, leading to multiorgan dysfunction secondary to chronic low-shear nonpulsatile pulmonary blood flow and central venous hypertension. Although blood viscosity increases exponentially in this low-shear environment, the role of shear-dependent (“non-Newtonian”) blood viscosity in this pathophysiology is unclear. We studied three-dimensional (3D)-printed Fontan models in an in vitro flow loop with a Philips 3-T magnetic resonance imaging (MRI) scanner. A 4D flow phase-contrast sequence was used to acquire a time-varying 3D velocity field for each experimental condition. On the basis of blood viscosity of a cohort of patients who had undergone the Fontan procedure, it was decided to use 0.04% xanthan gum as a non-Newtonian blood analog; 45% glycerol was used as a Newtonian control fluid. MRI data were analyzed using GTFlow and MATLAB software. The primary outcome, power loss, was significantly higher with the Newtonian fluid [14.8 (13.3, 16.4) vs. 8.1 (6.4, 9.8)%, medians with 95% confidence interval, P < 0.0001]. The Newtonian fluid also demonstrated marginally higher right pulmonary artery flow, marginally lower shear stress, and a trend toward higher caval flow mixing. Outcomes were modulated by Fontan model complexity, cardiac output, and caval flow ratio. Vortexes, helical flow, and stagnant flow were more prevalent with the non-Newtonian fluid. Our data demonstrate that shear-dependent viscosity significantly alters qualitative flow patterns, power loss, pulmonary flow distribution, shear stress, and caval flow mixing in synthetic models of the Fontan circulation. Potential clinical implications include effects on exercise capacity, ventilation-perfusion matching, risk of pulmonary arteriovenous malformations, and risk of thromboembolism. NEW & NOTEWORTHY Although blood viscosity increases exponentially in low-shear environments, the role of shear-dependent (“non-Newtonian”) blood viscosity in the pathophysiology of the low-shear Fontan circulation is unclear. We demonstrate that shear-dependent viscosity significantly alters qualitative flow patterns, power loss, pulmonary flow distribution, shear stress, and caval flow mixing in synthetic models of the Fontan circulation. Potential clinical implications include effects on exercise capacity, ventilation-perfusion matching, risk of pulmonary arteriovenous malformations, and risk of thromboembolism.

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Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

Cited by 22 publications

References 88 publications

Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation—Initial Results From a Prospective Study

Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation—Initial Results From a Prospective Study

Appropriate heart rate during exercise in Fontan patients

A 4D flow MRI evaluation of the impact of shear-dependent fluid viscosity on in vitro Fontan circulation flow

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