Aortic Arch Syndrome with Special Reference to Pulseless Disease and Its Variants

Abstract

(1) Out of 14 cases of aortic arch syndrome experienced during the last 11 years, 13 were diagnosed as pulseless disease described by Shimizu and Sano, reflecting the high frequency of this disease in Japan.(2) Six cases of atypical coarctation of the aorta seen during the same period of time were also analysed. The pathogenesis of atypical coarctation was interpreted to be identical to that of pulseless disease, based on the comparison of sex and age distribution, several laboratory data and autopsy findings between the both conditions.(3) It was recommended to describe these apparently different conditions under a single term, for example panaortitis syndrome, and to divide them into subtypes such as aortic arch type, abdominal aorta type and extensive type according to the localization of the lesions.(4) The prognosis of these patients seemed in general to be not unfavorable despite the persistence of disability of varying degrees.