Myocardial perfusion, function and exercise tolerance after the arterial switch operation

Objectives. This study was conducted to determine the prevalence of myocardial perfusion abnormalities at rest and exercise and to assess exercise capacity in children after the arterial switch operation. Background. There have been sporadic reports of myocardial ischemia or sudden death in children after the arterial switch operation for transposition of the great arteries, possibly related to inadequate coronary perfusion due to kinking or stenosis of the translocated coronary arteries. Methods. Myocardial perfusion at rest and peak exercise was assessed using the scintigraphic agent technetium-99m methoxyisobutyl isonitrile (sestamibi). Exercise capacity was determined with a modified Brace protocol. Ambulatory etectrocardiographic (ECG) Holter monitoring was performed. Ventricular function, contractility and wall motion were assessed echocardiographically. Results. Twenty-three children (aged 4.2 to 7.9 years) underwent evaluation. Abnormalities were found on the rest perfusion scans in 22 children (95.6%). The left ventricular myocardium was divided into 13 segments for analysis. Of 299 rest segments, 225 (75.3%) were normal, 11 (3.7%) showed mild defects, 45 (15%) moderate defects and 18 (6%) severe defects at rest. At peak exercise, 237 segments (79.3%) were normal, 24 (8%) showed mild defects, 33 (11%) moderate defects and 5 (1.7%) severe defects. Compared with rest studies, myocardial perfusion grade at exercise was unchanged in 246 segments (82.3%), improved in 42 (14%) and worsened in 11 (3.7%). All patients had normal exercise tolerance without symptoms or ischemic ECG changes. No ventricular tachycardia was seen on Holter monitoring. All patients had a shortening fraction ≥27%. Left ventricular contractility was normal in 12 children in whom it was assessed. Regional wall motion was normal in 17 children with adequate echocardiographic images for this analysis. Conclusions. Myocardial perfusion scan abnormalities assessed by technetium-99m sestamibi are common after an arterial switch operation. These abnormalities are of uncertain clinical significance and generally lessen with exercise. The normal exercise tolerance without symptoms or ECG changes suggests that myocardial perfusion is adequate during the physiologic stress of exercise in children up to 8 years after an arterial switch operation.