Currently, aortic aneurysm is the only phenotype listed by OMIM under MYH11. Similarly, homozygous variants in FBN2 and KRIT1, linked to autosomal dominant congenital contractural arachnodactyly and cerebral cavernous malformations 1, respectively, inherited from healthy heterozygous parents were identified in patients with arthrogryposis and NIHF, respectively. Here, FBN2 is linked to aortic aneurysm.