Two Coronary “Orphan” Diseases in Search of Clinical Consideration: Coronary Syndromes X and Y

We set out to describe the clinical characteristics of patients presenting with acute or stable coronary syndromes and no stenosis in epicardial coronaries. Although the existence of patients who experience typical angina and who have intact epicardial coronaries is well accepted, the pathophysiology of cardiac ischemia in this setting remains poorly understood. In typical coronary syndrome X, it is believed that at least two components play a role: the first is the incapacity of coronary resistance vessels to adapt to situations of increased blood demand, resulting in demand ischemia; the second is an inappropriate transduction or generation or pain stimuli within the central nervous system. These two mechanisms concur to determine episodes of precordial pain and electrocardiogram (ECG) evidence of ischemia during exercise. In contrast, the coronary slow-flow phenomenon, or syndrome Y, is an angiographic finding that is characterized by delayed progression of the contrast medium during coronary angiography. Although the mechanism of this phenomenon remains largely unknown, it has been proposed that it might depend on the presence of inappropriately high resting coronary resistances, causing reduced blood flow and therefore low-flow ischemia and unstable angina. Importantly, the prognosis of many of the patients presenting with coronary slow-flow does not appear to be favorable, with recurrence of acute coronary syndromes and life-threatening arrhythmias. In the present article, we revise the current evidence regarding these two phenomena, and propose that syndrome Y should be considered a separate clinical entity.