Korean J Leg Med.  2018 Feb;42(1):26-32. 10.7580/kjlm.2018.42.1.26.

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

Affiliations
  • 1Department of Forensic Medicine, Seoul National University College of Medicine, Seoul, Korea. sdlee@snu.ac.kr
  • 2Dongguk University Medical School, Gyeongju, Korea.
  • 3Medical Examiner's Office, National Forensic Service, Wonju, Korea.
  • 4Institute of Forensic Science, Seoul National University College of Medicine, Seoul, Korea.

Abstract

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.

Keyword

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

MeSH Terms

Atherosclerosis
Autopsy
Cause of Death
Coronary Disease
Coronary Vessels
Death, Sudden
Death, Sudden, Cardiac*
Fibromuscular Dysplasia*
Heart
Humans
Middle Aged
Prevalence
Reference Values
Resuscitation
Shoulder
Tunica Intima

Figure

  • Fig. 1. Gross findings of the heart and the coronary arteries of the deceased. (A,B) The heart was of normal weight without any abnormal features. (C-E) In the cross-sections of the major coronary arteries, the lumens were narrowed by wall thickening and there were no atherosclerotic changes (C, right coronary artery; D, anterior descending branch of the left coronary artery; E, circumflex branch of the left coronary artery).

  • Fig. 2. Histologic findings of coronary arteries. (A) The cross-section of the coronary artery shows circumferential thickening of the vascular wall (H&E stain, ×4). (B) The intimal layer which showed neither noticeable inflammation nor lipid accumulation contributed mainly to the thickening (H&E, ×40). (C, D) Elastin stain highlighted the internal elastic lamina and both fragmentations and duplications of the internal elastic lamina were found (C, elastin stain, ×100; D, elastin stain, ×200). These findings were consistent in all three major coronary branches.


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