Basso C, Morgagni G L, Thiene G
Department of Pathology, University of Padua Medical School, Italy.
Heart. 1996 May;75(5):451-4. doi: 10.1136/hrt.75.5.451.
Spontaneous coronary artery dissection is a rare cause of acute myocardial ischaemia. Eight consecutive fatal cases which occurred in women aged 34-54 years (mean 43) are described. The dissection involved the left anterior descending coronary artery in four, the left main trunk in two, the right coronary artery in one, and both left anterior descending and circumflex arteries in one. The clinical presentation was sudden death in six cases, and acute myocardial infarction in two. Diagnosis was made at necropsy in every case but one, in which coronary dissection was diagnosed during life by selective coronary angiography. The only ascertained risk factor was hypertension in one patient; none of the women was in the puerperium, and Marfan syndrome was excluded in all. Histology showed a haematoma between the coronary tunica media and adventitia, that flattened and occluded the lumen; a coronary intimal tear was detected in only two cases. Unusual histological findings were cystic medial necrosis in one case, eosinophilic inflammatory infiltrates in four, and angiomatosis of the tunica adventitia in one. Patients dying of spontaneous coronary dissection are usually middle aged women, with no coronary atherosclerosis and apparently no risk factors. Spontaneous coronary artery dissection is unpredictable, and sudden death is the usual mode of clinical presentation. Prompt diagnosis and life saving treatment is far from being achieved.
自发性冠状动脉夹层是急性心肌缺血的罕见病因。本文描述了8例连续发生的致命病例，患者为年龄在34至54岁（平均43岁）的女性。夹层累及左前降支冠状动脉4例，左主干2例，右冠状动脉1例，左前降支和左旋支动脉均受累1例。临床表现为6例猝死，2例急性心肌梗死。除1例在生前通过选择性冠状动脉造影诊断为冠状动脉夹层外，其余病例均在尸检时确诊。唯一确定的危险因素是1例患者患有高血压；所有女性均非产褥期，且均排除马凡综合征。组织学检查显示冠状动脉中膜和外膜之间有血肿，使管腔变平并闭塞；仅2例检测到冠状动脉内膜撕裂。不寻常的组织学表现为1例囊性中膜坏死，4例嗜酸性炎症浸润，1例外膜血管瘤病。死于自发性冠状动脉夹层的患者通常为中年女性，无冠状动脉粥样硬化且显然无危险因素。自发性冠状动脉夹层不可预测，猝死是常见的临床表现形式。目前远未实现及时诊断和挽救生命的治疗。
