A dipyridamole-induced Tl-201 perfusion abnormality was evaluated from its clinical features, echocardiography and myocardial histopathology in 39 patients with hypertrophic cardiomyopathy (HCM). From the findings of Tl-201 emission computed tomography (ECT), subjects were divided into three groups: group 1 (n = 16) which did not show a perfusion abnormality in the hypertrophic region; group 2 (n = 12) which showed a perfusion defect on the initial image with complete redistribution on the delayed image; and group 3 (n = 11) which showed a persistent perfusion defect-this group included most patients who revealed that group 2 showed a marked asymmetrical septal hypertrophy and an incidental obstructive pattern, and that group 3 had a significantly dilated left ventricular diastolic dimension and a decreased percentage of fractional shortening. Group 3 also showed frequent ventricular tachycardia and a familial history of cardiomyopathy. As for the myocardial biopsy findings, group 3 had significantly advanced myocardial fibrosis, the percentage being 6.03.1% in group 1; 5.52.5% in group 2; and 11.93.4% in group 3. Thus, it was concluded that the persistent perfusion defect on dipyridamole stress Tl-201 ECT testing is an important finding corresponding to the advanced clinical and pathological aspects of HCM.