Yelbuz Talât Mesud, Wessel Armin, Kirby Margaret L
Kinderklinik Medizinische Hochschule Hannover Abt. Pädiatrie III, Pädiatrische Kardiologie and Intensivmedizin, Carl-Neuberg-Str. 1, 30623 Hannover, Germany.
Z Kardiol. 2004 Aug;93(8):583-94. doi: 10.1007/s00392-004-0107-z.
Most congenital cardiovascular malformations have their origins during early morphogenesis, and some forms of adult-onset cardiovascular disease also arise during embryonic development. Conotruncal heart defects comprise a major category of congenital heart disease and are found in children with a relative high frequency. These defects are associated with a high mortality risk in utero, and after postnatal surgical repair; embryologically they are linked with dextroposed aorta, which is an anomaly of the ventricular outflow tract with malalignment of the great arteries. The etiology and pathogenesis of dextroposed aorta is not known but is thought to be due to abnormal looping and/or incorrect "wedging" of the outflow tract (i.e., wedged positioning of the aorta between the atrioventricular valves) during early heart development. We have studied the morphology and visual development of the embryonic heart in an animal model of dextropsed aorta in a series of experiments to determine possible mechanisms for dextropositioning of the aorta. At this, we have employed besides established methods for analysis of anatomy and pathology (morphological studies, cardiac morphometry, histology, scanning electron microscopy and immunhistochemistry) also new imaging techniques (videocinephotography and time-lapse studies with a digital high-speed video camera, confocal and scanning electron microscopy, optical coherence tomography (OCT) and magnetic resonance microscopy (MRM) for 3D reconstruction of the heart) to achieve a better visualization of normal and pathological changes during heart development. The paper at hand summarizes the results of these studies.
大多数先天性心血管畸形起源于早期形态发生阶段,某些形式的成人期心血管疾病也在胚胎发育期间出现。圆锥动脉干心脏缺陷是先天性心脏病的主要类型,在儿童中相对高发。这些缺陷在子宫内以及出生后手术修复后都伴有较高的死亡风险;从胚胎学角度来看,它们与右位主动脉有关,右位主动脉是心室流出道的一种异常,伴有大动脉排列不齐。右位主动脉的病因和发病机制尚不清楚,但被认为是由于心脏早期发育过程中流出道异常成环和/或不正确的“楔入”(即主动脉楔入房室瓣之间的位置)所致。我们在一系列实验中,对右位主动脉动物模型的胚胎心脏形态和视觉发育进行了研究,以确定主动脉右位的可能机制。为此,除了采用已有的解剖学和病理学分析方法(形态学研究、心脏形态测量、组织学、扫描电子显微镜和免疫组织化学)外,我们还采用了新的成像技术(电影摄影以及使用数字高速摄像机进行延时研究、共聚焦和扫描电子显微镜、光学相干断层扫描(OCT)和磁共振显微镜(MRM)用于心脏的三维重建),以便更好地观察心脏发育过程中的正常和病理变化。本文总结了这些研究结果。