Microcirculation and microstructure of coronary beds in Kawasaki Disease

我们遇到了9例川崎病患者，运动负荷试验发现缺血，但冠状动脉造影没有狭窄病变。为明确川崎冠状动脉血流储备减少的发病机制，我们对8例冠状动脉血流储备减少的患者进行了冠状动脉床和心肌病变的超微结构检查，与6例冠状动脉储备未降低的患者相比。患病时间为4个月至15年。所有患者均存活，但15年后仍有心肌和血管的持续性损害。超微结构上，心肌改变是非特异性的，表现为肥大和变性，细胞核奇异，肌原纤维排列紊乱，线粒体聚集异常。血管病变包括微动脉瘤形成，内皮细胞突起和坏死，以及毛细血管和小动脉中的血小板血栓形成。在本研究中，我们强调了血管病变具有进行性的潜在危险性，这是导致川崎病冠状动脉血流储备降低和持续性心肌病变的重要因素之一，尽管这种疾病在活动期没有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.