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[右冠状动脉起源于左冠窦异常。青少年猝死的一个可能原因]

[Anomalous origin of the right coronary from the left sinus of Valsalva. A possible cause of juvenile sudden death].

作者信息

Maresi E, Orlando E, Becchina G, Milone L, Procaccianti P, Campesi G

机构信息

Istituto di Anatomia Patologia, Università di Palermo.

出版信息

G Ital Cardiol. 1993 Oct;23(10):995-1003.

PMID:8174867
Abstract

Anomalous origin of the right coronary artery from the left sinus of Valsalva is a rare congenital anomaly classified as a "minor" anomaly of no clinical importance. Recently, manifestations of myocardial ischemia (angina pectoris, myocardial infarction, nonfatal ventricular fibrillation, sudden death) have been described in patients with this anomaly in the absence of atherosclerosis or other. Sudden death occurs frequently in symptomatic patients and rarely in asymptomatic patients (sudden unexpected death). In this study we report two cases of juvenile sudden death observed in asymptomatic patients with anomalous origin of the right coronary artery from the left sinus of Valsalva. In both cases the sudden death was exertion-related. In case 1 the coronary anomaly was the cause of death, since it was the only significant anatomic abnormality at necropsy; the microscopic findings revealed ischemic lesions only in the myocardium supplied by the anomalous right coronary artery. In case 2 the coronary anomaly was connected to other cardiac and non cardiac diseases (lymphocytic active myocarditis, chronic portitis, encephalitis, medullary adrenalitis). Since these morphologic lesions were extremely slight and there was no adrenal catecholamine damage in the myocardium, we consider negligible their possible role in determining death, which in this case was induced by the congenital coronary anomaly. The cardiac microscopic findings, also in this case, revealed ischemic lesions in the myocardium supplied by the anomalous right coronary artery.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

右冠状动脉起源于左冠状动脉窦是一种罕见的先天性异常,被归类为无临床意义的“轻微”异常。最近,已有该异常患者在无动脉粥样硬化或其他病变情况下出现心肌缺血表现(心绞痛、心肌梗死、非致命性室颤、猝死)的报道。猝死在有症状患者中频繁发生,在无症状患者中则很少见(意外猝死)。在本研究中,我们报告了两例右冠状动脉起源于左冠状动脉窦的无症状青少年猝死病例。两例猝死均与运动有关。病例1中,冠状动脉异常是死亡原因,因为尸检时它是唯一显著的解剖学异常;显微镜检查结果显示,仅在异常右冠状动脉供血的心肌中发现缺血性病变。病例2中,冠状动脉异常与其他心脏和非心脏疾病(淋巴细胞性活动性心肌炎、慢性门静脉炎、脑炎、肾上腺髓质炎)有关。由于这些形态学病变极其轻微,心肌中也没有肾上腺儿茶酚胺损伤,我们认为它们在决定死亡方面的可能作用可忽略不计,该病例的死亡是由先天性冠状动脉异常引起的。在该病例中,心脏显微镜检查结果同样显示,异常右冠状动脉供血的心肌存在缺血性病变。(摘要截断于250字)

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