Spontaneous aortic dissection due to cystic medial degeneration. Report of a sudden death case and literature review

A 32 years old woman collapsed during work, after complaining for several minutes of intense diffuse abdominal pains. An emergency team arrived on the spot succeded in reconverting a ventricular fibrillation and rushed the patient to an Emergency unit. Upon admission the patient was in shock (pulse 110/min, blood pressure 60/40 mmHg), extremely anxious, and continued to complain of intense epigastrial pains. A plain X ray and abdominal echography were irrelevant. Although the ECG suggested an acute myocardial infarction, the enzymatic markers of acute myocardial ischemia were uncharacteristic. The patient died 2 hours after admission, with the presumed diagnostic: "Acute myocardial infarction TOPOL I, possibly with spontaneous reperfusion. Cardiac arrest on ventilatory support". The autopsy revealed an almost circumferential aortic dissection initiated at the ostia of the left coronary artery, extending down to right common iliac artery. Microscopic examination revealed a myxoid degeneration between the disrupted elastic lamellae of the aortic media. The paper reviews the incidence of aortic spontaneous dissection, the pathogeny and associated genetic anomalies of aortic media degeneration.