Hutchins G M, Meredith M A, Moore G W
Hum Pathol. 1981 Mar;12(3):242-50. doi: 10.1016/s0046-8177(81)80125-6.
To examine the hypothesis that malpositions of cardiac ventricles could be explained by altered development of the interventricular septum, we studied hearts from the Johns Hopkins Hospital autopsy files with double inlet left ventricle (16 cases) or corrected transposition (nine cases). In double inlet left ventricle (16 cases) or corrected transposition (nine cases). In double inlet ventricle both atrioventricular valves connect normally developed and positioned right and left atria to a posterior morphologic left ventricle. In hearts with corrected transposition the atria are normally positioned and the morphologic right ventricle is on the left and is continuous with the anteriorly positioned aorta. The morphologic left ventricle is on the right, connected to the posteriorly positioned pulmonary trunk. Normal ventricular septation may be understood as arising from the mechanics of a spiral fold in the primary heart tube produced by the left interventricular sulcus. The ventral limb of the spiral induces the muscular interventricular septum while the dorsal limb becomes a component of the crista supraventricularis. We propose that double inlet left ventricle and corrected transposition are the result of minor deviations in the position of the interventricular sulcus on the primary heart tube. Double inlet left ventricle may develop from the formation of a closed unspiraled ring around the interventricular canal. Corrected transposition may result from a left interventricular sulcus whose ventral limb gives rise to a left sided crista supraventricularis, which determines in part the right ventricular morphology of the left sided ventricle. The dorsal limb spirals toward th atrioventricular canal, gives rise to a malpositioned interventricular septum, and displaces the embryonic trabeculated right ventricle to the left. The concept presented accounts for the morphologic findings characteristic of double inlet left ventricle and corrected transposition.
为检验心室位置异常可由室间隔发育改变来解释这一假说，我们研究了约翰霍普金斯医院尸检档案中的心脏，其中有双入口左心室（16例）或矫正性大动脉转位（9例）。在双入口左心室（16例）或矫正性大动脉转位（9例）中，在双入口心室中，两个房室瓣将正常发育且位置正常的右心房和左心房连接至后方形态学上的左心室。在矫正性大动脉转位的心脏中，心房位置正常，形态学上的右心室在左侧，并与前方位置的主动脉相连。形态学上的左心室在右侧，与后方位置的肺动脉干相连。正常的心室分隔可理解为由左心室沟在原始心管中产生的螺旋折叠机制所致。螺旋的腹侧肢诱导出肌性室间隔，而背侧肢则成为室上嵴的一个组成部分。我们提出，双入口左心室和矫正性大动脉转位是原始心管上室间沟位置的微小偏差所致。双入口左心室可能由围绕室间管形成一个闭合的非螺旋环发展而来。矫正性大动脉转位可能源于左心室沟，其腹侧肢产生左侧室上嵴，这部分决定了左侧心室的右心室形态。背侧肢向房室管螺旋，产生位置异常的室间隔，并将胚胎期有小梁的右心室向左移位。所提出的概念解释了双入口左心室和矫正性大动脉转位的形态学特征。
