Do Familial Aortic Dissections Tend to Occur at the Same Age?

Abstract

Familial dissections occur earlier than sporadic dissections. Dissections cluster by age in families, and age at onset can predict the age of other dissectors. This finding argues for consideration of prophylactic resection of an aneurysm in family members approaching the age at onset of prior thoracic aortic dissection.

The River of no Return: Acute Aortic Dissection with Catastrophic Outcome

“…Women with AD are generally older (67-years) and have a more delayed presentation [6,11]. Younger patients are often having history of hypertension, connective tissue disease, or anatomical abnormalities (Marfan syndrome, Ehlers-Danlos syndrome, Turners syndrome with coarctation of the aorta with bicuspid aortic valves) [12][13][14][15]. Inflammatory diseases…”

Aortic Dissection

“…However both men and women can develop these conditions at any age, but in females the outcome is worse. On the other hand, familial aortic dissections occur in younger patients as compared to sporadic aortic dissection [6].…”

The role of genetic testing in the prevention of acute aortic dissection

“…Since the identification of the FNB1 gene causing Marfan syndrome in 1991 1,3 and the familial nature of TAA in the 1990s 3,4 our knowledge of the genetic aspects of TAA has made great strides forward. The number of genes associated with TAA is growing steadily every year and to date 29 genes have been associated with TAA.…”

“…A positive familial history for TAAD impacts on all these aspects. 1,2,62 The estimated annual growth rate for TAA is, on average, 0.10 cm/year 63 with a faster growth rate for the descending thoracic aorta compared with the ascending (0.19 cm/year vs. 0.07 cm/year). 64 In FTAAD, the annual growth rate has been estimated to be 0.21 cm/year, 2 more than twice the rate of sporadic cases.…”

“…Clinicians are, however, aware of certain associations which can lead to an early diagnosis of TAA. The importance of a positive familial history is overwhelming [1][2][3][4] and there are other clinical signs which can lead to diagnosis of a silent TAA. 5 Once the diagnosis of TAA is made, the appropriate timing of prophylactic surgery is determined primarily by aortic size criteria.…”