Familial Aortic Aneurysms: History Of Rupture

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Familial inheritance of aortic aneurysms is well known. Screening of the general population at large for abdominal aortic aneurysms (AAA) has been shown to be cost-ineffective. Our efforts are now directed at managing the “Family Aorta” and prevention of ruptures.
Objective:
To determine the rupture rate of AAA in families with a history of AAA. Methods: Since 1994, 649 patients with abdominal aortic aneurysms have been followed. Of this population, 528 are male (81.4 %) and 121 are female (18.6%).
Family histories were documented by direct questioning. All patients were asked if they had documentation or knew of any family members, either first or second degree, with an aneurysm. In addition, we documented those family members with a history of a ruptured AAA.
Results:
A family history of an aneurysm occurred in 105/649(16.2%) patients. Of these, abdominal (n=82), cerebral (n=8), thoracic (n=3), femoral (n=2), arch (n=1) and multiple assorted aneurysms (n=9). Within the 105 familial aortas, 30 ruptures (28.6%) were reported. Within the abdominal aortic group (n=82), 17.07% occurred in mothers (n=14) (ruptured n=5) , 13.41% in fathers (n=11) (ruptured n=5), 25.61% among brothers (n=21) (ruptured n=7), 6.1% in sisters (n=5) (ruptured n=2), 31.71% among multiple family members (n=26) (ruptured n=7), 3.66% among cousins (n=3) with no ruptures, 1.22% with a child (n=1) (ruptured n=1), and 1.22% in aunts (n=1) (ruptured n=1).
Conclusions:
Family history of ruptured AAA is significant. The vast majority of these families have first degree inheritance patterns with the strongest relationship amongst brothers. With a significant degree of family history of ruptures, we suggest screening of all first degree family members for aneurysmal disease.