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Blunted peripheral blood supply and underdeveloped skeletal muscle in Fontan patients: The impact on functional
Aida Luiza Ribeiro Turquetto1, Marcelo Rodrigues Dos Santos1, Ana Luiza Carrari Sayegh1
1Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil.
Insights
Fontan patients exhibit underdeveloped skeletal muscles and reduced strength, linked to poor blood flow and exercise capacity. This study highlights the impact of Fontan surgery on peripheral circulation and muscle development.
Area of Science:
- Cardiovascular Physiology
- Pediatric Cardiology
- Exercise Science
Background:
- Fontan-associated circulatory changes can compromise peripheral blood flow and skeletal muscle function.
- Suboptimal cardiac output in Fontan patients may lead to impaired peripheral circulation.
Purpose of the Study:
- To investigate forearm blood flow (FBF), thigh muscle cross-sectional area (CSA), and functional capacity in stable Fontan patients.
- To compare circulatory and muscular parameters in Fontan patients versus healthy controls.
Main Methods:
- Utilized venous occlusion plethysmography and magnetic resonance imaging (MRI) for FBF and thigh CSA assessment.
- Conducted maximal cardiopulmonary exercise testing, measured muscle sympathetic nerve activity (MSNA), and assessed handgrip strength and 6-minute walk distance.
Main Results:
- Fontan patients demonstrated significantly reduced FBF, forearm vascular conductance (FVC), thigh CSA, peak VO2, walk distance, and handgrip strength compared to controls.
- Elevated MSNA and norepinephrine levels were observed in Fontan patients.
- Reduced FVC and stroke volume were independently associated with decreased thigh CSA, while thigh CSA correlated with peak VO2.
Conclusions:
- Fontan operation is associated with underdeveloped skeletal muscle, reduced strength, impaired peripheral blood supply, and diminished exercise capacity.
- These findings underscore the long-term physiological adaptations and limitations in Fontan survivors.
Background:
Changes in circulatory physiology are common in Fontan patients due to suboptimal cardiac output, which may reduce the peripheral blood flow and impair the skeletal muscle. The objective of this study was to investigate the forearm blood flow (FBF), cross-sectional area (CSA) of the thigh and functional capacity in asymptomatic clinically stable patients undergoing Fontan surgery.
Methods:
Thirty Fontan patients and 27 healthy subjects underwent venous occlusion plethysmography, magnetic resonance imaging of the thigh musculature and maximal cardiopulmonary exercise testing. Muscle sympathetic nerve activity (MSNA), norepinephrine measures, cardiovascular magnetic resonance, handgrip strength and 6-minute walk test were also performed.
Results:
Fontan patients have blunted FBF (1.59 ± 0.33 vs 2.17 ± 0.52 mL/min/100 mL p < 0.001) and forearm vascular conductance (FVC) (1.69 ± 0.04 vs 2.34 ± 0.62 units p < 0.001), reduced CSA of the thigh (81.2 ± 18.6 vs 116.3 ± 26.4 cm2p < 0.001), lower peak VO2 (29.3 ± 6 vs 41.5 ± 9 mL/kg/min p < 0.001), walked distance (607 ± 60 vs 701 ± 58 m p < 0.001) and handgrip strength (21 ± 9 vs 30 ± 8 kgf p < 0.001). The MSNA (30 ± 4 vs 22 ± 3 bursts/min p < 0.001) and norepinephrine concentration [265 (236-344) vs 222 (147-262) pg/mL p = 0.006] were also higher in Fontan patients. Multivariate linear regression showed FVC (β = 0.653; CI = 0.102-1.205; p = 0.022) and stroke volume (β = 0.018; CI = 0.007-0.029; p = 0.002) to be independently associated with reduced CSA of the thigh adjusted for body mass index. The CSA of the thigh adjusted for body mass index (β = 5.283; CI = 2.254-8.312; p = 0.001) was independently associated with reduced peak VO2.
Conclusion:
Patients with Fontan operation have underdeveloped skeletal muscle with reduced strength that is associated with suboptimal peripheral blood supply and diminished exercise capacity.
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