Management hereditärer thorakaler Aortenerkrankungen (HTAD)

Hereditary thoracic aortic diseases (HTAD) are an underappreciated problem in vascular surgery. First, HTAD are often considered a “rarity”, although they account for approximately 20% of all thoracic aortic aneurysms and aortic dissections. Second, HTAD are often recognized too late, although specific warning signs provide clear evidence of Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS), vascular Ehlers-Danlos syndrome (vEDS), or nonsyndromic HTAD (ns-HTAD). Third, HTAD are often treated as common degenerative or atherosclerotic aortic diseases (DATAD), although HTAD require specific open surgical repair (OSR) and thoracic endovascular aortic repair (TEVAR). This article summarizes the established knowledge of the epidemiology, diagnosis, and repair of HTAD that is necessary for optimal treatment.