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Abstract Background Coronary atherosclerosis and calcific aortic valve disease (CAVD) have similar risk factors and disease mechanisms. However, the presence of coronary atherosclerosis does not warrant the development of CAVD. Therefore, proving the association between the degree of coronary artery calcium (CAC) and the progression of CAVD could improve follow-up and treatment strategies. Purpose To explore the assoication between the degree of CAC and the onset and progression of CAVD from a single-centre registry of coronary CT angiographic and serial echocardiographic examinations. Methods We retrospectively included 2,898 patients who underwent coronary CT angiography and serial echocardiographic examinations at intervals of every 6 months or more. The CAC was divided into 4 groups: 0, 1–99, 100–399 and ≥400. The progression of CAVD was defined in two ways: Progression 1 as at least one grade progression (e.g. from mild aortic stenosis (AS) to moderate or severe AS), Progression 2 as at least moderate AS at follow-up. We used multivariable logistic regression analyses to assess the factors associated with the progression of CAVD. Results Among the 2,898 patients, CAC 0, 1–99, 100–399 and ≥400 groups were 1,122, 786, 556 and 434, respectively. As CAC increased, those have more comorbidities and worsened echocardiographic parameters associated with left ventricular systolic and diastolic function. At the initial CAVD grade, patients with at least mild AS tended to increase with increasing CAC (P < 0.001). During a median follow-up of 3.2 years (IQR, 1.8–5.0 years), 101 patients (3.5%) experienced Progression 1 and 24 patients (0.8%) suffered Progression 2. There was a statistically significant increased risk of Progression 1 in CAC 100–399 and ≥400 groups than CAC 0 and 1–99 groups (P < 0.001). In Progression 2, the CAC ≥400 group was remarkably progressed than other groups (P < 0.001). The CAC had a fair ability to predict risk of Progression 2 (area under the curve = 0.737) and the cut-off value was 133. In multivariable logistic regression, age, CAC ≥400 (adjusted odds ratio [aOR], 2.55; 95% confidence interval [CI], 1.19–5.46; P = 0.016), body mass index and peak aortic jet velocity were associated with Progression 1. In Progression 2, CAC ≥400 (aOR, 44.5; 95% CI, 1.09–1810; P = 0.045) and peak aortic jet velocity were significant determinants. Conclusion Coronary artery calcium was significantly associated with the onset and progression of calcific aortic valve disease. Patients with CAC 100 or more need to consider screening and follow-up for AS, especially CAC 400 or more is crucial for the progression to significant AS.KM curve of Progression 1-free survival KM curve of Progression 2-free survival