Sudden Cardiac Death due to Coronary Fibromuscular Dysplasia: Case Report and Literature Review

Korean J Leg Med 2018;42:26-32

Department of Forensic Medicine, Seoul National University College of Medicine, Seoul, Korea,

Dongguk University Medical School, Gyeongju, Korea,

Medical Examiner's Office, National Forensic Service, Wonju, Korea,

Institute of Forensic Science, Seoul National University College of Medicine, Seoul, Korea

E-mail.: sdlee@snu.ac.kr

Fibromuscular dysplasia (FMD) of the coronary artery is a rare cause of sudden cardiac death; however, its prevalence and fatality may have been overlooked so far. A 47-year-old man complained of pain in his back and shoulder and became unconscious. Despite resuscitation, he died 3 hours after symptom onset. The heart weight was in the normal range; however, all three major coronary arteries showed intimal thickening without atherosclerosis or inflammatory cell infiltration. Fragmentations and duplications of the internal elastic lamina which are histologic features of intimal fibroplasia, a focal-type FMD, were observed. The prevalence of coronary FMD remains unknown, although it may be related to spontaneous coronary artery dissection and sudden death. The histopathologic confirmation of coronary FMD and exclusion of other possible coronary diseases through autopsy are essential to reveal the nature of the disease and therefore apply the information in dealing with legal problems after death.

Key Words: Fibromuscular dysplasia; Coronary vessels; Tunica intima; Cause of death; Sudden cardiac death