997.
A newly recognized type of complete d-transposition of the great arteries is presented. The aortic valve was posterior and inferior to the pulmonary valve in 4 postmortem cases. Deficiency of the subaortic muscular conus permitted tenuous aortic-tricuspid fibrous continuity in 3 of 4 cases and tenuous aortic-mitral fibrous continuity in all by means of a high ventricular septal defect. The crista supraventricularis (parietal band) was entirely above the morphologically left ventricle and was much shorter than normal (average length 3 mm, normal average length 7 mm).
The morphogenesis of these transpositions cannot be explained by the straight truncal septum hypothesis because the truncal septum in these cases was spiral, not straight. These new findings can readily be explained by the conal growth hypothesis, and they indicate the desirability of a literal (accurate) definition of transposition of the great arteries: aorta arising above the morphologically right ventricle and pulmonary artery originating above the morphologically left ventricle. Malpositions of the great arteries include transposition, double-outlet right ventricle, double-outlet left ventricle and anatomically corrected malposition (“transposition”).
Transposition of the great arteries is a relation, not an entity. It is 3 different entities in terms of conal malformations (on the basis of these 4 cases plus a control study of 100 unselected autopsy cases of transposition, accurately defined): (1) subaortic conus with pulmonary-mitral fibrous continuity, 92 percent; (2) subaortic and subpulmonary (bilateral) conus without semilunar-atrioventricular fibrous continuity, 8 per cent; and (3) markedly foreshortened subpulmonary conus with tenuous aortic-atrioventricular fibrous continuity, as in these 4 cases, much less than 1 percent. 相似文献