Three-stage extensive aortic substitution in a young man with a variant of uncertain significance in myosin heavy chain 11 gene.

BackgroundNon-syndromic hereditary aortic diseases are caused by mutations in genes encoding for connective tissue components, involving structural integrity of aortic tissue. Amongst them, MYH11 gene mutation is a known causative factor for aortic diseases, especially in young patients.Research DesignWe report the case of a 37 years-old male which came to our attention for an acute DeBakey type I aortic dissection. In three different operations he was submitted to a total aortic substitution with a Bentall operation, a Frozen Elephant Trunk and a Thoracoabdominal Aortic open repair. During the last two operations particular technical tips, which are reported in this work, have been adopted to optimize surgical strategy.