Exercise testing has become an essential tool in the management of patients with CAL. In addition to its ability to assess exercise limitation objectively, it has usefulness in detecting the presence or absence of associated disease processes, in assessing the response to therapies, in allowing assessment of the importance of psychological factors in exercise limitation, and in guiding prescription for exercise rehabilitation programs. Although much is known about the clinical usefulness of exercise testing in this disease, and much has been learned about how this disease functionally impairs the exercise capacity of the patient, additional study is necessary to appreciate fully the physiologic abnormalities demonstrated by patients with CAL during exercise.