TY - JOUR
AR - JICOA-2020-2-103
TI - Morphological Characteristics of Aortic Stenosis in Familial Hypercholesterolemia and Non-Familial Hypercholesterolemia in the Elderly
AU - Hatsue, Ishibashi-Ueda
AU - Mariko Harada-Shiba, 
AU - Michio Noguchi, 
AU - Mika Hori, 
AU - Naotaka Ohta, 
AU - Tomoyuki Fujita, 
AU - Tsutomu Tomita, 
JO - Journal of Integrative Cardiology Open Access
PY - 2020
DA - Mon 13, Apr 2020
SN - 2674-2489
DO - http://dx.doi.org/10.31487/j.JICOA.2020.02.03
UR - https://www.sciencerepository.org/morphological-characteristics-of-aortic-stenosis_JICOA-2020-2-103 
KW - Aortic stenosis, familial hypercholesterolemia, calcific aortic stenosis, histology
AB - Background: Patients with familial hypercholesterolemia are known to have an extremely high risk of
coronary artery disease owing to high levels of low-density lipoprotein-cholesterol since birth. In addition,
aortic stenosis among patients with familial hypercholesterolemia has also been reported besides coronary
disease. The aim of this study was to characterize the histopathological differences in excised aortic valves
for aortic stenosis between patients with familial hypercholesterolemia and non-familial
hypercholesterolemia.
Subjects and Methods: Six familial patients (3 homozygous, 3 heterozygous familial hypercholesterolemia
patients), and 18 non-familial hypercholesterolemia patients underwent pathological and
immunohistochemical examinations for aortic valves macroscopically and microscopically at aortic valve
replacement surgery for stenosis.
Results: Our study revealed that calcification of aortic valves among homozygous hypercholesterolemia
showed a much milder degree than those of non-familial patients. Moreover, the age at surgery for stenosis
in the case of homozygotes was significantly less than that of heterozygous hypercholesterolemia and nonfamilial hypercholesterolemia patients. In addition, CD68-positive macrophages infiltrated the aorta side
(fibrosa) in all familial hypercholesterolemia patients. However, the macrophage accumulation in the aortic
valves of non-familial hypercholesterolemia patients was recognized in the middle layer (spongiosa) near
calcification and left ventricular side (ventricularis) of the aortic valves.
Conclusion: Lipid is one of the important factors for aortic valve fibrosis and stenosis in
hypercholesterolemia. This study suggested that the non-familial atherosclerotic aortic stenosis in the
elderly is qualitatively different from aortic valves in familial hypercholesterolemia, primarily owing
to calcification resulting from age and long-term degeneration and inflammatory responses.