Key result
Atypical aortic coarctation and pulseless disease share common pathogenesis, supporting classification as panaortitis syndrome.
Why the study?
Aortic arch syndrome and its variants, including pulseless disease and atypical coarctation, require clarification regarding their relationship and classification.
Observational (n=20)
No
Hypothesis-generating for unified aortitis nomenclature; does not yet alter clinical diagnostic criteria.
(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.
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UEDA et al. (1963) conducted an observational in Aortic arch syndrome and atypical coarctation of the aorta (n=20). Pulseless disease and atypical coarctation of the aorta was evaluated on Clinical manifestations and pathogenesis. Atypical coarctation of the aorta and pulseless disease share essentially the same pathogenesis and should be classified under a single term such as panaortitis syndrome.
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