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.
Abstract

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