Exercise Capacity in Congenital Heart Disease: The Role of Muscle Metabolism

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 60

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

Patients and controls:
- age <8 and >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)

Study & Design

Study Type: Observational non invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>o Quadriceps phosphocreatine concentration (PCr) at rest and during progressive<br /><br>exercise (exercise intensities 0-100% of workload corresponding to VO2max)<br /><br>o Quadriceps inorganic phosphorus concentration (Pi) at rest and during<br /><br>exercise (exercise intensities 0-100% of workload corresponding to VO2max)<br /><br>o Quadriceps pH at rest and during exercise (exercise intensities 0-100% of<br /><br>workload corresponding to VO2max)<br /><br>o Post-exercise recovery rate of quadriceps PCr concentration<br /><br>o Post-exercise recovery rate of quadriceps Pi concentration<br /><br>o Post-exercise recovery rate of quadriceps pH</p><br>

Secondary Outcome Measures