Wistuba I, Arcil G, Farrú O, Chuaqui B
Abteilung für Pathologische Anatomie, Medizinische Hochschule, Päpstlichen Katholischen Universität Santiago, Chile.
Pathologe. 1993 May;14(3):127-30.
A sporadic case of idiopathic asymmetric heart hypertrophy in a newborn infant is reported. Despite drug therapy the baby died in progressive heart failure at 23 days of age. At necropsy there was cardiac hypertrophy with features similar to those of the usual asymmetric from observed in adults. On gross examination the myocardium of the ventricular septum and the free wall of the left ventricle showed a disorganized structure. Microscopically, the changes consisted of an abnormal arrangement of muscle cells and muscle bundles with areas of hypertrophied myofibers. In agreement with other authors myocardial disorganization ("disarray") is interpreted as a form of dysplasia, hypertrophy being a secondary phenomenon. The hypothesis that these abnormalities represent the persistence of the embryonic myocardial structure is discussed. The pathogenetic significance of focal myocardial dysplasias is apparently related to the amount of myocardium involved.
报告了一例新生儿特发性不对称性心脏肥大的散发病例。尽管进行了药物治疗,婴儿仍在23日龄时死于进行性心力衰竭。尸检时发现心脏肥大,其特征与在成人中观察到的常见不对称性心脏肥大相似。大体检查显示,室间隔和左心室游离壁的心肌结构紊乱。显微镜下,变化包括肌细胞和肌束排列异常以及肌纤维肥大区域。与其他作者一致,心肌结构紊乱(“排列紊乱”)被解释为发育异常的一种形式,肥大是继发现象。讨论了这些异常代表胚胎心肌结构持续存在的假说。局灶性心肌发育异常的发病机制意义显然与受累心肌的量有关。