Le risque familial de l'anévrisme de l'aorte abdominale et ses conséquences pour l'organisation d'un dépistage sélectif

Aneurysm of the abdominal aorta (AAA) is multifactorial disease related to acquired factors (arteriosclerosis) and genetic factors (collagen anomalies) with, in some cases, a familial component. The incidence of familial AAA was evaluated in 324 patients in 313 families who underwent surgery for AAA. A family survey was used to determine whether members of the operated patients had AAA or had died due to ruptured aneurysms. There were 276 cases (including 12 females) with sporadic AAA and 81 cases (including 5 females) who were members of families with at least one other member having AAA. Comparison between sporadic AAA (SAAA) and familial AAA (FAAA) demonstrated the following differences: 1) diagnosis was made earlier in FAAA (p < 0.05) than in SAAA (64.1 versus 66 years of age respectively), 2) patients with FAAA were younger at the time of rupture (65.4 versus 75.1 years, p < 0.001), 3) the incidence of rupture was greater in FAAA than in SAAA (32.4% versus 8.7% p < 0.001), and finally 4) the relative risk of developing AAA was 18 times greater in members of a family with AAA than in the reference population. Early screening with ultrasonic examinations in at least patient's brother's over 50 years would not be an unjustified expense. The frequency of later examinations would depend on the size of the aorta initially observed. If FAAA is evidenced, under normal conditions, corrective surgery should be proposed earlier than for sporadic AAA.