This study reveals that the Type B aortic dissection (TBAD) is commonly thought of as a sporadic event. However, an increasing body of data has suggested that genetic factors can influence TBAD. Our aim was to determine the prevalence of heritable TBAD, defined as either syndromic TBAD or nonsyndromic familial TBAD and to detail the natural history and long-term clinical outcomes compared with patients with “sporadic” TBAD without an identified syndrome or family history.

The clinical records of 389 patients with TBAD who had presented to a single health care system from 1995 to 2017 were reviewed. A family history was obtained by interview and/or medical record review. Syndromic TBAD was defined as TBAD in patients with Marfan, Loeys-Dietz, or vascular Ehlers-Danlos syndrome. Nonsyndromic familial TBAD was defined as a family history of aortic or arterial aneurysm or dissection and/or sudden death in a first- or second-degree relative in the absence of a known syndrome. Patients with syndromic and nonsyndromic familial TBAD were compared with patients with sporadic TBAD in terms of the comorbid conditions, aortic repair, and mortality.

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