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Aerobic fitness is an important determinant of overall health. Higher aerobic fitness has been associated with many health benefits. Because myocardial ischemia is rare in children, indications for exercise testing differ in children compared with adults. Pediatric exercise testing is imperative to unravel the physiological mechanisms of reduced aerobic fitness and to evaluate intervention effects in children and adolescents with a chronic disease or disability. Cardiopulmonary exercise testing includes the measurement of respiratory gas exchange and is the gold standard for determining aerobic fitness, as well as for examining the integrated physiological responses to exercise in pediatric medicine. As the physiological responses to exercise change during growth and development, appropriate pediatric reference values are essential for an adequate interpretation of the cardiopulmonary exercise test.