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Right ventricular (RV) performance during supine bicycle exercise was evaluated by gated equilibrium nuclear angiography in 19 clinically well children with d-transposition of the great arteries (d-TGA), 6.4 +/- 2.7 years after Mustard's operation. Comparisons were made between rest and peak exercise. The mean resting ejection fraction was 44 +/- 12% (range 30-75%) and was unchanged at peak exercise. Eight children had a normal ejection fraction response, whereas 11 children had either no increase or a decrease in ejection fraction. Relative end-diastolic volumes decreased from resting values in all patients who had an abnormal ejection fraction response. Among patients whose ejection fraction increased, the end-diastolic volume increased in three, decreased in four and was unchanged in one at peak exercise. Heart rate increased 84% (range 52-135%) and systolic blood pressure increased 16% (range 0-28%) at peak exercise. There was no correlation between exercise response and age at surgery or interval since surgery. These data indicate that clinically well children after Mustard's procedure may have abnormal right ventricular function under stress, raising concerns about the ability of the right ventricle to function as the systemic ventricle.
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