congenital heart disease decreased exercise tolerance
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: Patients with pulmonary arterial hypertension: - Between 8 and 18 years of age - Diagnosis confirmed at the UMC Groningen Patients with a Fontan circulation: - Between 8 and 18 years of age - Diagnosis confirmed by the UMC Groningen - Successfully undergone a Fontan procedure - Stable haemodynamic condition Patients with tetralogy of Fallot: - Between 8 and 18 years of age - Diagnosis confirmed by the UMC Groningen - Successfully undergone procedure to repair the abnormalities;Healthy subjects between 8 and 18 years of age, without significant cardiac defect or known comorbidity affecting exercise tolerance.;All parents and subjects from 12 y.a.o.: Written informed consent
Exclusion criteria
Exclusion criteria: Patients and controls: - age 18 - not familiar with the Dutch language - ineligible to perform an exercise test - with contra-indications for 31P/1H MRS examination - (suspected) pregnancy - mental retardation - with comorbidity affecting exercise tolerance (Anaemia, musculoskeletal injury) - being under examination for non-diagnosed disease at the time of investigation Patients: - with severe complications due to cardiomyopathy/arrhythmia - with exacerbation of disease at time of investigation - with a pacemaker implanted - with severe complications due to epilepsy - with musculoskeletal disease (i.e. muscular dystrophy)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| o Quadriceps phosphocreatine concentration (PCr) at rest and during progressive exercise (exercise intensities 0-100% of workload corresponding to VO2max) o Quadriceps inorganic phosphorus concentration (Pi) at rest and during exercise (exercise intensities 0-100% of workload corresponding to VO2max) o Quadriceps pH at rest and during exercise (exercise intensities 0-100% of workload corresponding to VO2max) o Post-exercise recovery rate of quadriceps PCr concentration o Post-exercise recovery rate of quadriceps Pi concentration o Post-exercise recovery rate of quadriceps pH | — |
Secondary
| Measure | Time frame |
|---|---|
| n.a. | — |
Countries
Netherlands