22(3):225227, 2001.
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).
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-
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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.
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.
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