SUMMARYIt has been suggested that mitral annular calcification (MAC) may be a manifestation of generalized atherosclerosis. However, how the incidence and extent of coronary artery disease (CAD) are affected by the coexistence of carotid atherosclerosis (CAS) in patients with versus without MAC have not yet been studied.We studied 101 patients with echocardiographic MAC and 52 controls without MAC to investigate the clinical impact of CAS on the frequency and severity (defined as the number of obstructed vessels) of CAD in patients with MAC. Carotid Doppler ultrasonographic examination was performed on all patients before coronary angiography. In patients with both MAC and CAS, the incidences of CAD and multivessel disease (≥ 2 vessel or left main coronary artery disease) were significantly higher than in the control group with CAS alone (91% versus 68%, P = 0.008 and 76% versus 44%, P = 0.004, respectively). On the other hand, among study and control patients without CAS, although the frequencies of CAD and multivessel disease were higher in patients with MAC, interestingly, the differences were not statistically significant (37% versus 58% and 15% versus 26%, respectively, P > 0.05 for both). Stepwise multiple logistic regression analysis revealed that CAS (P < 0.001), MAC (P < 0.01) and, to a limited extent hypertension (P = 0.054), were independent predictors for the presence of CAD.In conclusion, the coexistence of CAS is more important in patients with MAC than in those without as it provides valuable information about the incidence and severity of underlying CAD. In cases with MAC but without CAS, MAC could be caused by factors other than atherosclerosis. (Jpn Heart J 2004; 45: 603-611)