Exercise performance is decreased in patients with Thalassemia major (TM), but the relative impact of anemia and iron overload on exercise capacity is unknown. We assessed the cardiopulmonary function of 71, well-transfused TM patients via graded treadmill exercise stress test. All patients underwent MRI of the heart, pancreas, and liver and diagnostic phlebotomy. Patients ranged in age from 13 to 46 years of age. Fifteen patients were excluded from analysis due to submaximal effort. Mean Vo 2 max was 83.0% of predicted and was limited by abnormal cardiovascular mechanisms, consisting of a decreased O 2 pulse (86.6% of predicted) in men and decreased maximum heart rate (HR) response (85% of predicted) in women. Patients with hemoglobin less than 12 g/dL had lower O 2 pulse and Vo 2 max, regardless of sex. Cardiac iron was negatively associated with maximum HR response and Vo 2 max (r 2 5 0.10 and 0.08, respectively, P < 0.05). Vo 2 max was correlated with cardiac R2*, hs-CRP, sex and hemoglobin in decreasing strength of association. In thalassemia, exercise performance is limited by impaired stroke-volume reserve in men and blunted HR response in women. Iron toxicity may be mediated through vascular inflammation and direct modulation of HR response to exercise. Am. J.

Summary This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32%, and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for airways obstruction, there were indicators of small airways disease and air trapping. PFTs did not correlate with somatic iron burden, blood counts or haemolysis. Restrictive lung disease was associated with inflammation. We conclude that TM patients have pulmonary abnormalities consistent with small airways obstruction. Restrictive disease and impaired diffusion are less common.

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