Cardiopulmonary stress testing in children with sickle cell disease who are on long-term erythrocytapheresis.

The purpose of the study was to assess the feasibility and safety of symptom-limited cardiopulmonary stress testing (CPST) in children with sickle cell disease (SCD), who are on long-term erythrocytapheresis. Maximal symptom-limited CPST was performed in 16 children with SCD who were maintained on long-term erythrocytapheresis and the exercise response in this patient cohort was compared with those of a healthy control population. All patients completed the CPST without any complications. Twelve patients with SCD had significant reduction in aerobic capacity [peak oxygen consumption (VO2) <80% predicted]. No patient demonstrated evidence of myocardial ischemia. In summary, symptom-limited CPST can be performed safely in a subgroup of children with SCD and can yield valuable clinical information.