Van Praagh R, Durnin R E, Jockin H, Wagner H R, Korns M, Garabedian H, Ando M, Calder A L
Circulation. 1975 Jan;51(1):20-31. doi: 10.1161/01.cir.51.1.20.
Two autopsy-proved cases are presented of a rare form of congenital heart disease, anatomically corrected malposition (S, D, L). Anatomically corrected malposition means that despite the abnormal relationship between the great arteries, the aorta arises nonetheless above the anatomically left ventricle and the pulmonary artery originates above the anatomically right ventricle. (S, D. L) briefly indicates the segmental set or combination: situs solitus of viscera and atria (S), ventricular D-loop (D), AND L-MALPOSITION OF THE GREAT ARTERIES (L). These are the first cases of anatomically corrected malposition (ACM) in whom the presence of a subaortic muscular conus only has been documented pathologically. This established that ACM can occur with a subaortic conus, as well as with a bilateral conus. From the diagnostic standpoint, the distributions or epicardial courses of the coronary arteries are recommended as a helpful approach to ventricular identification. This diagnostic method has widespread angiocardiographic and surgical applications. Symbolic terminology, exemplified by anatomically corrected malposition (S, D. L) is brief and accurate. This approach may conveniently be applied to many other forms of comples congenital heart disease, and it considerably facilitates computer data processing.
本文报告了两例经尸检证实的罕见先天性心脏病病例,即解剖矫正型大动脉错位(S,D,L)。解剖矫正型大动脉错位是指尽管大动脉之间关系异常,但主动脉仍起自解剖学上的左心室上方,肺动脉起自解剖学上的右心室上方。(S,D,L)简要表示节段组合:内脏和心房位置正常(S)、心室D袢(D)以及大动脉左旋(L)。这是首例经病理证实仅存在主动脉下肌性圆锥的解剖矫正型大动脉错位(ACM)病例。这证实了ACM可伴有主动脉下圆锥以及双侧圆锥。从诊断角度来看,推荐冠状动脉的分布或心外膜走行作为识别心室的有用方法。这种诊断方法在心血管造影和手术中有广泛应用。以解剖矫正型大动脉错位(S,D,L)为例的符号术语简洁准确。这种方法可方便地应用于许多其他复杂先天性心脏病形式,并且极大地促进了计算机数据处理。