In a very recent paper, Kent et al. suggested a NOTCH1-dependent mechanism that produces stenotic, insufficient and/or calcified aortic valves with a rare aneurysm, and a NOTCH1-independent mechanism that produces highly penetrant ascending aortic aneurysm (AscAA) in the presence of a non-calcified and often normally functioning BAV [29]. The gene discussed is NOTCH1; the disease is Ascending tubular aorta aneurysm.