Regional myocardial perfusion abnormalities commonly occur during exercise in patients with hypertrophic cardiomyopathy (HCM). Exercise 201Tl myocardial scintigraphy has provided a noninvasive means of identifying myocardial perfusion abnormalities in patients with HCM. On the other hand, apical hypertrophic cardiomyopathy (APH) is reported as a subtype of HCM. Whether APH is essentially equal to HCM or not is controversial. To assess myocardial ischemia in patients with APH, we studied 28 patients with APH, with exercise 201Tl SPECT. Myocardial perfusion images were obtained immediately after submaximal exercise and again after a 3-hour delay. Regional perfusion defects during exercise were identified in 19 of the 28 patients (68%) with APH. Complete reversible defects were observed in 15 (79%) patients with APH. Although perfusion defects were present in all regions of the left ventricle in patients with HCM, they were present only in the apical region in patients with APH. Thus, reversible 201Tl perfusion abnormalities commonly occur during exercise in patients with APH as well as in patients with HCM.