MYOCARDIAL-INFARCTION BY SPONTANEOUS RUPT URE OF THE CORONARY-ARTERIES IN TYPE-IV EHLERS-DANLOS SYNDROME

Acute myocardial infarction with spontaneous coronary dissection was f atal in a 33-years-old man. Autopsy and family history revealed type I V Ehlers-Danlos syndrome, In this disease, conjunctive tissue is fragi lized 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 sho uld be entertained in young people with no atherogenous risk factor an d an acute coronary disorder since peripheral skin and joint syndromes may be discreet or missing. Treatment is difficult in ease of spontan eous coronary dissection. Thrombolysis is not possible and diagnosis m ay require angiography which is in itself dangerous due to the fragili ty of the coronary vessels.