The American Journal of Forensic Medicine and Pathology

22(3):225227, 2001.

2001 Lippincott Williams & Wilkins, Inc., Philadelphia

Sudden Death Related to Small Coronary Artery Disease

K. Michaud, M.D., N. Romain, M.D., C. Brandt-Casadevall, M.D., and P. Mangin, Ph.D.

Fibromuscular dysplasia was first described by Leadbetter and Burkland in 1938 as a disease of renal arteries,
after it was also observed in cervical and other arteries. It
is a nonatheromatous, noninflammatory disease of the arteries with segmental stenosis (1). According to James
(2), the focal fibromuscular dysplasia of small coronary
arteries rarely extends more than a few millimeters along
the course of an artery. The typical lesion is often entirely
within the tunica media, including both fibrous and muscular components, and the histologic organization is so
disorderly that it is considered a dysplastic process. A
uniform histologic classification of fibromuscular dysplasia of coronary arteries does not exist (1).
The physiologic consequence of focal fibromuscular
dysplasia with the synergistic combination of local spasm
has been discussed by James and Bruschke (3), who reported that for a small coronary artery with 50% narrowing of the radius of the lumen, only a 9% decrease of the
external radius by spasm is sufficient to obliterate the
lumen completely. An obstruction of the arteries supplying the conduction system may have caused the electrical
instability of the heart with arrhythmia and sudden death.
Thickening of the small intramural coronary arteries
has been reported in some cases of sudden death (4), but
there are very few reports of death in young persons related to focal fibromuscular dysplasia of coronary arteries supplying the conduction system (1,5,6).

Two cases of sudden death of young people in apparently

good health are reported. The only pathologic change
found was a fibromuscular dysplasia of the artery supplying the conduction system of the heart with an important
narrowing of the lumen and strong thickening of the arterial wall. The first case was of a 12-year-old girl who died
suddenly while skiing; the second was of a 32-year-old
man who died while talking to his wife. No other pathologic changes were found at autopsy, and the results of
toxicologic analysis were negative. There was no individual or family history of cardiac diseases. These cases illustrate the importance of an analysis of the conduction
system, including examination of the intramural coronary
arteries supplying the conduction system.
Key Words: Sudden deathFibromuscular dysplasia
Conduction systemCoronary artery.

CASE REPORTS
Case 1
An apparently healthy 12-year-old girl died suddenly
while skiing. No family history of cardiac disease was reported. The decedent was 1.40 m tall and weighed 32 kg.
The heart was mildly enlarged and weighed 150 g. A
slight hypertrophy of the left ventricle was noticed. We
also observed signs of mitral valve prolapse: the posterior
leaflet of the mitral valve was slightly dome-shaped and
shortened, and the left atrium was mildly enlarged, with
the endocardium diffusely thickened and whitened.
The conduction system was studied as follows: first,
five blocks containing the atrioventricular node, the bun-

Manuscript received November 30, 2000; accepted January

5, 2001.
From the Institut Universitaire de Mdecine Lgale, Lausanne, Switzerland.
Address correspondence and reprint requests to K. Michaud,
Institut Universitaire de Mdecine Lgale, Bugnon 21, 1005
Lausanne, Switzerland.

225

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K. MICHAUD ET AL.

FIG. 1. Summit of the ventricular septum in case 1, with

the atrioventricular node artery adjacent to the central fibrous body.

dle of His, and the proximal part of the right and left bundle branches were obtained and then cut along planes
perpendicular to the anulus of the valve from anterior to
posterior, and at least two sections were prepared from
each block. The sections were stained with hematoxylin
and eosin, and some were stained with Masson trichrome
and elastic van Gieson.
Microscopy revealed focal fibromuscular dysplasia of
the atrioventricular artery with focal very narrowed
lumen (Figs. 1 and 2), destruction of the internal elastic
lamina (Fig. 3), and focal fibrosis of the summit of the
ventricular septum, suggesting previous ischemia. There
were also some contraction bands of the working myocardium. The arterial walls of other organs were normal.
The results of toxicologic analysis (immunoassays and
gas chromatography/mass spectrometry drug screening)
were negative.

FIG. 2. Strong thickening of the arterial wall and narrowing

of the lumen in case 1.
Am J Forensic Med Pathol, Vol. 22, No. 3, September 2001

FIG. 3. Destruction of the internal elastic lamina in case 1

(elastic van Gieson stain).

Case 2
A 34-year-old man with no previous medical problems
died suddenly while talking to his wife. He was 191 cm
tall. His heart weighed 480 g, and there was a slight dilatation of both ventricles. The coronary arteries were
normal except for a small plaque of the left coronary
artery but without any evidence of thrombosis or significant stenosis (less than 25%). The valves were normal.
Microscopic examination demonstrated a focal strong
thickening of an artery situated in the bundle of His with
a very severe focal narrowing of the lumen (Figs. 4 and 5)
with essentially fibrous component. The same artery a
few millimeters away had normal lumen and wall without
any pathologic changes. Parts of the conduction fibers
were replaced by fatty cells. The results of toxicologic
analysis were negative.
DISCUSSION
In forensic medicine, many cases of sudden and unexpected death must be investigated. The majority of

FIG. 4. Focal obstruction of the artery situated in the bundle of His, in case 2.

SUDDEN DEATH RELATED TO SMALL CORONARY ARTERY DISEASE

FIG. 5. Strong thickening of the arterial wall and narrowing

of the lumen in case 2.

cases of witnessed collapse or of an individual found

under apparently natural circumstances can be resolved
after autopsy when a natural cause appears, for example, internal hemorrhage. Some cases of sudden death,
usually those occurring during stress or physical activity, can be compatible with sudden cardiac death. Some
of them can be explained by obvious cardiac pathologic
changes found at autopsy, such as atherosclerotic coronary disease, valve disease, or myocardial disease, or
after histologic examination. However, even after a
complete autopsy with toxicologic analysis, some
deaths remain unexplained. Moreover, it is well known
that disturbances of cardiac rhythm are a common
pathophysiologic mechanism responsible for sudden
death. In some cases, such as in long QT and Brugada
syndromes, these disturbances are only functional and
do not involve morphologic changes. In others, a congenital or acquired lesion affecting the conduction system of the heart can be found, which can explain the
clinical signs.
The common features in our two cases were sudden
unexpected death, a morphologically normal heart showing only a strong focal narrowing of the artery supplying
the conduction system, no other pathologic lesions, and
negative results of toxicologic analysis.
Although another origin of death cannot be excluded,
as for example rhythm disturbances of physiologic origin, it is important to notice that gene defects in ion channels, as in long QT syndrome or Brugada syndrome, are
frequently related to electrocardiographic abnormalities
revealed earlier in the subjects life or in family members.
In the cases discussed here, there was no individual or
family history of cardiac disease.

227

Moreover, the mitral valve prolapse observed in the

first case may not have been the only cause of the death,
the more so because mitral valve prolapse (floppy mitral
valve) with a minor degree of prolapse is very common in
the normal population, as is shown by echocardiographic
surveys of young subjects (7). However, Burke et al. (8)
reported that arterial dysplasia in mitral valve prolapse
may contribute to sudden cardiac death mediated by ventricular fibrosis.
Burke et al. (9) compared the atrioventricular node
artery in 27 patients who died of unexplained sudden
death with 17 normal control subjects and demonstrated
a quantitative difference between the degree of atrioventricular node artery narrowing in both groups. Moreover,
the authors observed that sudden death was often associated with exercise and a family history of unexplained
sudden death.
Therefore, considering the physiologic consequences
of a local spasm in a small coronary artery with a narrowed lumen, it can be concluded that in our cases a focal
narrowing of the arteries supplying the conduction system of the heart might have led to lethal arrhythmia.
In addition, these two cases illustrate the importance of
an analysis of the conduction system, including an examination of the intramural coronary arteries supplying the
conduction system, particularly in cases of sudden and
unexpected deaths of young people who die without any
obvious case of death.
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3. James TN, Bruschke AVG. The spectrum of diseases of
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4. Burke AP, Virmani R. Intramural coronary artery dysplasia
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5. Anderson R, Bowie J, Dempster AG, et al. Sudden death
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Am J Forensic Med Pathol, Vol. 22, No. 3, September 2001