Hort W, Reinecke P
Pathologisches Institut der Universität Düsseldorf.
Z Kardiol. 1989;78 Suppl 7:27-32.
The heart of neonates and infants with marked hypoplastic left- or right-heart-syndrome contains--despite the very low weight in the hypoplastic parts--myocytes of approximately the same size as in the non-hypoplastic ventricular wall. This means that the number of cells is not constant, because in contrast to the regularly developed right and left ventricular walls, the total number of myocytes in the hypoplastic parts is significantly lower. Hypertrophy of the myocardium in congenital heart disease shows histological and ultrastructural findings that are similar to those found in other forms of myocardial hypertrophy. Hypertrophy is reversible after successful surgical correction. However, regression is often incomplete, especially if surgery is delayed until adolescent or adult life. During this time scar formation in the hypertrophic ventricular wall may develop. In addition to these scars, also postoperative scar formation may contribute to the origin of arrhythmias. In hearts with cyanotic and non-cyanotic vitia distances between capillaries and capillary lengths are approximately the same as in normal hearts. Obviously, these parameters are genetically determined.
患有明显左心或右心发育不全综合征的新生儿和婴儿的心脏，尽管发育不全部分的重量极低，但其中的心肌细胞大小与非发育不全的心室壁中的心肌细胞大致相同。这意味着细胞数量并不恒定，因为与正常发育的右心室和左心室壁不同，发育不全部分的心肌细胞总数明显较少。先天性心脏病中的心肌肥大在组织学和超微结构上的表现，与其他形式的心肌肥大相似。成功进行手术矫正后，肥大是可逆的。然而，恢复往往不完全，尤其是如果手术推迟到青少年或成年期。在此期间，肥厚的心室壁可能会形成瘢痕。除了这些瘢痕外，术后瘢痕形成也可能导致心律失常的发生。在有青紫型和非青紫型缺损的心脏中，毛细血管之间的距离和毛细血管长度与正常心脏大致相同。显然，这些参数是由基因决定的。
