Aortic valvular stenosis with coarctation of the aorta, with special reference to the development of aortic stenosis upon congenital bicuspid valves.

The combination of coarctation of the aorta and aortic valvular stenosis is not well known, although the association of coarctation with aortic incompetence is generally recognized and usually attributed to an incompetent bicuspid aortic valve. Abbot (1928), in her review of coarctation, did not discuss its association with aortic valvular stenosis. However, she reported four cases of sub-aortic stenosis occurring with coarctation (an incidence of 2%), and Taussig (1947) regards this combination as occurring so frequently as to constitute a clinical entity. Wood (1950) states briefly that with coarctation " aortic or sub-aortic stenosis is by no means rare." Reifenstein et al. (1947) in their series of 104 post-mortem cases of coarctation record 11 with " calcific aortic stenosis " (each of which they regard as being due to rheumatic disease), but they do not enlarge upon this association. Recently Jacobson et al. (1953) have collected 14 reported cases of " calcific aortic stenosis " complicating coarctation, and add one further case; they comment on the surprising rarity of bicuspid aortic valves, which they found in only 3 of 14 necropsy cases [one (Gilbert et al., 1950) lacks post-mortem confirmation], and on the rarity of calcific aortic valves. Five cases of aortic stenosis complicating coarctation have recently come to our attention. This has prompted a review, from which we have collected 24 cases with necropsy confirmation of this combination.