Recent clinical and laboratory observations indicate that coronary artery spasm may play a role in the pathophysiology of ischemic heart disease. The majority of patients with ischemic heart disease have coronary atherosclerosis. The prevalence of coronary artery spasm in these patients is unknown. However, current evidence suggests that patients with rest angina have a higher incidence of coronary artery spasm than do patients with reproducible effort angina. Coronary artery spasm may initiate or contribute to acute myocardial infarction, but recent evidence obtained in patients undergoing thrombolytic therapy during the early phases of myocardial infarction suggests that it is not a common occurrence. Although numerous examples of ventricular tachycardia and ventricular fibrillation occur during episodes of coronary artery spasm, the incidence of coronary artery spasm in association with sudden death is unknown. Provocative testing with ergonovine maleate reveals that the highest incidence of provocable coronary artery spasm is found in patients with rest angina. In patients with the syndrome of variant angina, coronary artery spasm is nearly always provocable.