A new article from the Montalcino Aortic Consortium (MAC), “PRKG1 pathogenic variants cause thoracic aortic dissection with minimal aortic dilation: Insights from the UTHealth Houston multidisciplinary aortic and vascular disease conference,” highlights that recurrent PRKG1 pathogenic variants (p.Arg177Gln/p.Arg192Gln) are associated with heritable thoracic aortic disease (HTAD) characterized by early, often fatal aortic dissections that may occur without significant aortic enlargement. The findings underscore the aggressive nature of PRKG1-related disease and the importance of gene-based surveillance and early surgical intervention to prevent dissection.