Four patients had the clinical features of 'ampulla cardiomyopathy', consisting of acute-onset transient left ventricular apical akinesis with basal normokinesis, normal coronary angiogram, ST-segment elevation and subsequent giant T wave inversion, which mimicked acute coronary syndrome, the onset of which occurred shortly after extreme mental stress. Myocardial necrosis was minimal, although 2 patients showed elevated serum catecholamine levels in the acute phase. Each patient underwent serial cardiac radionuclide single-photon emission computed tomography of myocardial functional sympathetic innervation, fatty acid metabolism and perfusion using I-123-metaiodobenzyl-guanidine (MIBG), I-123-beta-metyl-iodophenyl pentadecanoic acid (BMIPP) and thallium-201 (201Tl), respectively. In the acute phase, MIBG and BMIPP imaging showed an uptake defect in the apical region, whereas 201Tl uptake was mildly decreased. When assessed semi-quantitatively, the MIBG images had higher defect scores from the acute phase throughout the year of observation compared with BMIPP, and 201Tl. These observations suggest that the primary cause of ampulla cardiomyopathy is related to a disturbance of the cardiac sympathetic innervation.