Background: The exact etiology and prognostic significance of coronary artery ectasia (CAE) is still unknown. The presence of CAE is influenced by genetic factors and related to the presence of aneurysms in other vascular beds. Bicuspid aortic valve (BAV) disease is frequently accompanied by ascending aortic aneurysm. Since the aortic valve, the ascending aorta, and the proximal parts of the coronary arteries share a common embryonic origin, we hypothesized that CAE is associated with BAV disease. Methods: 181 patients with suspected aortic valve disease (n=101 BAV) underwent both cardiac magnetic resonance (CMR) imaging and coronary angiography. Eighty subjects with tricuspid aortic valve (TAV) disease were similarly studied and served as controls. The readers of the angiograms were blinded to valve type and clinical data. In order to confirm the association of CAE with BAV, the frequency of CAE was evaluated in an in-house BAV registry (n=500) and compared to the frequency of CAE in the German MI family study, in which the heritability of CAE was formerly established (899 with available coronary angiograms), as well as in an observational registry of “real-life patients” undergoing coronary angiography for clinically indicated reasons (n=3471). Furthermore the frequency of CAE was investigated in a subgroup of the KORA MI study, which is a population-based registry that comprises all hospitalized cases of acute non-fatal MI and coronary deaths occurring in inhabitants of a defined study region (n=403). Results: Compared to TAV disease, CAE occured twice as frequently in CMR confirmed BAV disease, (17.5% vs. 41.6%, p=0.0005). Ascending aortic aneurysm or ectasia was diagnosed in 60 subjects with BAV disease (59.4%), but CAE occurred similarly between subjects with (59.5%) and without (40.5%) ascending aortic pathology. The common appearance of CAE in patients with BAV could be independently confirmed in the BAV registry (38.9%), whereas CAE was found less frequently in family history positive MI patients (21.2%), and rarely in unrelated “real-life” catheterization patients (5.2%). Conclusion: To our knowledge, our data show for the first time that ectatic coronary artery disease is a common appearance of BAV disease with and without ascending aortic aneurysm.