Right ventricular systolic function in adolescents and young adults after Mustard operation for transposition of the great arteries.

This study evaluates long-term ( > 10 years since surgery) right ventricular (RV) systolic function in patients who had previously undergone intraatrial baffle surgery for transposition of the great arteries. Studies suggest these patients are clinically stable and lead satisfactory lifestyles, but long-term ventricular performance is not known. Radionuclide angiocardiography was used to estimate RV ejection fraction in 58 patients a mean of 14 years after the Mustard operation. Repeat studies were performed in 32 patients. The absolute RV ejection fraction of 0.53 +/- 0.10 in our patients did not differ from normal values. Nine patients had a value < 0.42, placing them > 2 SDs below normal. Repeat RV ejection fraction decreased from 0.54 +/- 0.11 to 0.51 +/- 0.11 (p < 0.1) in 32 patients, and > 0.10 in 6. Thus, RV ejection fraction was abnormal in 9 of 58 patients (16%) evaluated > 10 years after a Mustard operation. Repeat studies demonstrate worsening in at least 6 of 32 patients (19%). These postoperative Mustard patients require continued evaluation, even in the absence of overt symptomatology.