Andrews R E, Tulloh R M R, Anderson D R, Lucas S B
Guy's and St Thomas' Hospital, London, UK.
Heart. 2004 Apr;90(4):e17. doi: 10.1136/hrt.2003.018499.
A 20 month old child with hypoplastic left heart syndrome died suddenly from a massive myocardial infarction 15 months after a hemi-Fontan operation. This was confirmed at postmortem examination and histological examinations. The sites of surgical reconstruction were all in good condition, there were no gross anatomical coronary abnormalities, and the coronary ostia were unobstructed. On microscopy the internal coronary arteries had notable intimal and medial thickening with narrowing of the lumen, although no thrombotic occlusion was seen. To the authors' knowledge, this is the first published report of arteriosclerosis of the coronary arteries in hypoplastic left heart syndrome. It raises the question as to whether there may be a primary histological abnormality in some children with this condition or whether some mechanism of accelerated arteriosclerosis is at work.
一名患有左心发育不全综合征的20个月大儿童在进行半Fontan手术后15个月，因大面积心肌梗死突然死亡。尸检和组织学检查证实了这一点。手术重建部位均状况良好，没有明显的解剖学冠状动脉异常，冠状动脉开口也无阻塞。显微镜检查显示冠状动脉内膜和中膜显著增厚，管腔狭窄，尽管未见血栓性阻塞。据作者所知，这是左心发育不全综合征冠状动脉硬化的首例发表报告。这就提出了一个问题，即患有这种疾病的一些儿童是否可能存在原发性组织学异常，或者是否存在某种加速动脉硬化的机制在起作用。
