Catanese V, Venot P, Lemesle F, Delille F, Runge I, Kuchly B
Service de Réanimation médicale polyvalente-Urgences-SMUR, Centre Hospitalier de l'Agglomération montargoise, Amilly-Montargis.
Presse Med. 1995 Oct 7;24(29):1345-7.
Acute myocardial infarction with spontaneous coronary dissection was fatal in a 33-years-old man. Autopsy and family history revealed type IV Ehlers-Danlos syndrome. In this disease, conjunctive tissue is fragilized due to a deficit in type III collagen which leads to spontaneous ruptures in large and medium sized arteries. The present case is the first with a proven rupture of the coronary arteries. This disease should be entertained in young people with no atherogenous risk factor and an acute coronary disorder since peripheral skin and joint syndromes may be discreet or missing. Treatment is difficult in case of spontaneous coronary dissection. Thrombolysis is not possible and diagnosis may require angiography which is in itself dangerous due to the fragility of the coronary vessels.
一名33岁男性因自发性冠状动脉夹层导致急性心肌梗死死亡。尸检和家族史显示为IV型埃勒斯-当洛综合征。在这种疾病中,由于III型胶原蛋白缺乏,结缔组织变得脆弱,导致大中型动脉出现自发性破裂。本病例是首例经证实的冠状动脉破裂。对于没有动脉粥样硬化危险因素且患有急性冠状动脉疾病的年轻人,应考虑这种疾病,因为外周皮肤和关节综合征可能不明显或不存在。自发性冠状动脉夹层的治疗很困难。无法进行溶栓治疗,诊断可能需要血管造影,而由于冠状动脉血管脆弱,血管造影本身就有危险。