Functional Muscle Characteristics and Cardio-respiratory Interaction in Patients With Fontan Palliation
Condition: Patients
Study type: Interventional

Summary: 
      Congenital heart disease (CHD) is the most common congenital defect, affecting about 1% of
      newborns. Among adults surviving complex CHD, the cohort of survivors after the Fontan
      palliation for univentricular physiology is the most rapidly growing. Given their unique
      physiology without a pumping chamber supporting the pulmonary circulation, their exercise
      capacity as adults is often severely reduced. Therefore, patients with grown-up CHD typically
      need long-term expert medical care causing constantly increasing healthcare- related costs.
      Specific exercise regimes might offer a safe, efficacious and cost-effective alternative to
      pharmacologic interventions and surgery. The underlying mechanisms of impaired exercise
      capacity in Fontan patients are, however, not fully understood and consequently, training
      regimes specifically tackling the limiting factors cannot be developed. In patients with CHD,
      studies have suggested that exercise limitations are secondary to the cardiac defects,
      factors related to cardiac surgery, chronotropic incompetence and underlying lung disease.
      Similar to heart failure patients, exercise limitations could be traced back to insufficient
      respiratory and leg muscle functions and impaired cardiovascular regeneration processes
      possibly due to an altered stem cell number and function in the peripheral blood.

      The present study will investigate cardiac, respiratory and quadriceps muscle function at
      rest and during submaximal and maximal whole-body exercises. A special focus will be given on
      respiratory and quadriceps muscle strength, and proneness of these muscles to fatigue, which
      has not yet been objectively investigated. Moreover, different respiratory muscle training
      (RMT) protocols will be executed, to assess the safety of these interventions. Moreover, the
      current study will be the first to investigate the influence of exhaustive exercise protocols
      on the number and function of circulating stem and progenitor cells (CPCs) in Fontan
      patients. The number of these cell populations was shown to strongly correlate with long-term
      outcome and recovery in several diseases. This study aims to reveal whether Fontan patients
      show similarly alternated stem cell number and function in the peripheral blood, that likely
      result in impaired vascular regeneration processes and possibly also contribute to reduced
      exercise capacity.
    


Intervention name(s): Incremental cycling test (ICT); Constant load cycling test (CET); Incremental respiratory muscle test (IncRMET); Incremental quadriceps muscle test (IncQMT); Respiratory muscle training like interventions
Intervention type(s): Other; Other; Other; Other; Other
Intervention description(s): Incremental cycling test to volitional exhaustion; Constant load cycling test to volitional exhaustion.; Incremental respiratory muscle test to volitional exhaustion.; Incremental quadriceps muscle test to volitional exhaustion.; Three different respiratory muscle training like interventions that are usually used for respiratory muscle trainings.
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Primary purpose: Basic Science