Microcirculation and microstructure of coronary beds in Kawasaki Disease

我们遇到了9例川崎病患者，在运动负荷试验中发现缺血，但在冠状动脉造影中没有狭窄病变。9例患者中有8例冠状动脉血流储备减少，这是由于冠状动脉造影未显示的小冠状动脉扩张能力降低所致。为了阐明川崎病冠状动脉血流储备减少的发病机制，本研究对上述8例冠状动脉血流储备减少患者的心肌病变和冠状动脉床进行了超微结构检查，并与6例未发生冠状动脉储备减少的患者进行了比较。病程从4个月到15年不等。所有患者均存活，但15年后心肌和血管的损害仍持续存在。超微结构变化无特异性，心肌肥大变性，细胞核奇异，肌原纤维紊乱，线粒体聚集异常。血管病变包括微动脉瘤形成、内皮细胞突出和坏死、毛细血管和小动脉血小板血栓形成。间质水肿和纤维化也会出现。在本研究中，我们强调血管病变具有进行性的潜在危险，这是导致川崎病冠状动脉血流储备减少和持续心肌病变的重要因素之一，尽管在川崎病的活动期没有出现活动性炎症或其他证据。

We encountered 9 patients with Kawasaki disease with ischemic findings on exercise stress tests, but without a stenotic lesions on coronary arteriography. 8 out of these 9 patients had a reduced coronary flow reserve due to reduced dilatory capacity of the small coronary arteries that are not visualized by coronary arteriography.To clarify the pathogenesis of reduced coronary flow reserve in Kawasaki disease, in this research, we examined ultrastructually the myocardial lesions and coronary bed in 8 patients with reduced coronary flow reserve described above, in comparison with 6 patients without reduced coronary reserve. The duration of illness is from 4 months to 15 years. All patients are still alive, but the persistent damages of myocardium and vasculature are found even after 15 years. Ultrastructurally, the myocardial changes are non-specific, showing hypertrophy and degeneration with bizarre nuclei, disarranged myofibrils and aggregative mitochondria with abnormalities. The vascular lesions included microaneurysm formation, endothelial cell projection and necrosis, and platelet thrombosis in both capillary and arteriole. Interstitial edema and fibrosis are also encountered.In this study, we emphasized that the vascular lesions have a progressive potential danger, which are one of the important factors induced the reduced coronary flow reserve and the persistent myocardial lesions in Kawasaki disease, though there is no active inflammation or other evidence seen in active phase of this disease.