Functional assessment of ALCAPA syndrome by dobutamine stress thallium-201 SPECT and echocardiography.

Exercise 201TI SPECT has been used as a useful method for the assessment of patients with anomalous left coronary artery communicating to the pulmonary artery (ALCAPA syndrome). In this study, we described an adult patient with this anomaly who was evaluated by dobutamine stress testing in conjunction with simultaneous 201Tl SPECT and echocardiography before and after surgery. A large perfusion defect in the anterior wall, septum and apex was detected on the preoperative stress scan with partial reversibility on reinjection scan. Worsening of wall motion abnormalities in the septum and anterior wall was detected by stress echocardiography. In the studies performed 3 mo and 1 yr after reimplantation of the left coronary artery in the aorta, a smaller fixed perfusion defect in the anterior wall and apex was detected without reversibility. No stress-induced wall motion abnormalities were detected. Despite the improvement of perfusion, there was no improvement of regional or global left ventricular function at rest. We report that both dobutamine 201Tl SPECT and echocardiography were useful for the detection of reversible ischemia and for the assessment of the surgical outcome of an adult patient with ALCAPA syndrome.