[Aortic insufficiency and Takayasu disease]

Six cases of this rare association (7 to 10 p. 100 of Takayasu's disease) are reported. The authors also review 73 previously reported cases. The valvular lesion is usually detected secondarily during follow-up (2/3 of cases), but is observed at the same time as the arterial disease in about 1 out of 5 cases. In rare cases, it may be the presenting feature before the vascular disease becomes clinically apparent. Quantification of the regurgitation may be difficult because of stenosis of the thoracic aorta and the supra aortic vessels. In some cases it is severe and poorly tolerated but has no particular distinguishing features apart from the incidence of aortic wall calcification (ascending aorta to all of the aortic arch). Twenty five anatomical observations (operative or autopsy) are sufficiently well documented to show the mechanism of the aortic incompetence. It is caused by an inflammatory aortitis: valvular lesions were found in 2/3 of cases but other causes may be observed, dilatation of the aortic ring (1 out of 4 cases), disunion of the commissures (1 out of 4 cases) and changes in aortic compliance causing systolic hypertension. Aortic valve replacement is rare (11 cases including 3 personal cases) and sometimes completed by resection of an aneurysmal ascending aorta.