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Kei Takahashi, MD0.18 Histopathological analysis of sudden death adults with coronary artery aneurysms
FINANCIAL DISCLOSURE:No relevant financial relationship exists
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O-18
Histopathological analysis of sudden death adults with coronary artery aneurysms
Kei Takahashi1, Toshiaki Oharaseki1, Yuki Yokouchi1, Yasunori Enomoto1, , Kino Hayashi2, Kumiko Asakura2, Kazuyuki Saito2,3, Aya Takada2,4
1 Department of Pathology, Toho University Ohashi Medical Center2 Tokyo Medical Examiner's Office3 Department of Forensic Medicine, Juntendo University School of Medicine4 Department of Forensic Medicine, Saitama Medical University Faculty of Medicine
IKDS2015
0-4歳人口
10万対
第22回全国調査成績 日本川崎病グループ
0.0
50.0
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250.0
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2,000
4,000
6,000
8,000
10,000
12,000
14,000
16,000
~1964
1966
1968
1970
1972
1974
1976
1978
1980
1982
1984
1986
1988
1990
1992
1994
1996
1998
2000
2002
2004
2006
2008
2010
2012
male
female
incidence rate
Up to 1993年 122,237ptsSequela 3% 3,667pts
5% 6,112pts10% 12,224pts
Up to 1973年 5,450ptssequel 10% 545pts
Total Number of KD Patients 299,440ptsCardiovascular abnormalities in acute phase 9.3%Cardiovascular sequela 2.8%
【Aims】
To clarify the long-term changes in coronary artery lesions in Kawasaki disease (KD) by investigating autopsy cases of adults who had post-inflammatory coronary artery lesions.
【Materials】
Sixteen autopsy cases over 18 years of age, with1) a history of KD in infancy or childhood, or2) coronary artery aneurysms at autopsy despite no confirmed history of KD.
Age at death : 18 to 57 years old.
Males : Females = 11 : 5
1 18 F 165cm58Kg
Student 8yo: viral infection suspected, positive for anti-nuclear ab, collagen disease suspected 2013.4Rested in a sickbay owing to bad health and suddenly stopped and collapsed
2 19 M Student 10yo:fever of unknown origin 2000.1Collapsed suddenly while playing basketball in PE class
3 19 M 174cm73Kg
Student 3yo: KD under follow-up 1994.11 Complained about epigastralgia and collapsed suddenly in class a few days later
4 20 F 164cm53Kg
Office worker 5yo, 7yo: acute febrile exanthematous disease, 17yo: arrhythmia, under f/u 1984Was found lying in an office.
5 20 M Student 4yo: KD under f/u 1989.4Was quarreling on the road, then ran away with a bicycle. Suddenly stopped and collapsed.
6 24 F 160cm53Kg
Office worker Elementary student:Juvenile rheumatoid arthritis
2004.5Got a day off work for feeling unwell. Father took her to a hospital in the evening.
Suffered sudden cardiopulmonary arrest after vomiting and suffering convulsion.
7 25 M 171cm88Kg
Office worker 4yo: KD, f/u until 15yo 2013.11Was found lying on a road while jogging in a evening.8 27 M 173cm
59KgOffice worker childhood: slapped cheek disease 2013.4Was found lying on a road after drinking at mid-night.
9 33 M 184cm76Kg
none 25yo: integration disorder, under f/u 1998.2Was found lying in a bathroom at home at night10 34 M 175cm
90KgCarrier 1y6mo: KD, no CAL, f/u until15 yo 2011.1Complained about bad health in a morning and suddenly collapsed in a evening
11 40 M 174cm65Kg
Pipe fitter 20yo: hypertension, cardiac hypertrophy, 38yo: heart failure 1985.11Collapsed suddenly while working
12 40 F 160cm46Kg
Housekeeper 37yo: first attack of AMI, stent implantation in #6&7, repeated stent thrombosis (BMSx1, DESx2). 2006.2
Suffered sudden cardiopulmonary arrest after 5months of the last DES implantation
13 44 F good nutrition
Office worker KD (age unknown), Hashimoto disease (detail unknown)2009.12
Got on a bus after riding bicycle in a morning. deteriorated suddenly in a bus. Vf to stand still on EMS arrival.
14 45 M 188cm107Kg
Sheet-metal worker 2013.5Was found lying on a road in a morning after riding bicycle15 50 M 165cm
50KgElectrician 3yo: cyanosis, pertussis? , 44yo: alcohol abuse 2008.2
Worked as usual. Had difficulty in breathing while a passenger in a car and lost consciousness suddenly.
16 57 M 171cm 67Kg
Office worker infant: Rheumatic fever because of high fever 2004.1Sudden death (detail unknown)
Sudden death 16 ptsHistory of KD 5 ptsHistory of acute febrile disorder 6 ptsin childhoodPositive antinuclear antibody test 1 ptUnspecified 4 pts
Summary of the 16 autopsied casesAge at death 10s – 3 pts,
20s – 5 pts, 30s – 2 pts, 40s – 4 pts, 50s – 2 pts
【Observation items】Clinical CourseCoronary Arterial Lesions
Aneurysm 【+ / -】Size 【Diameter】Site 【LMT-LAD / LCX / RCA (segment)】Form 【Dilated AN / Recanalized AN】
Calcification 【Lamellar / Granular】Mural thrombosis 【+ / -】Atherosclerosis【AHA classification】Thrombotic occlusion 【+ / -】Plaque injury 【Erosion / Rupture】
Myocardium LesionsIschemic lesion 【+ / -, Site, AMI/ OMI】Interstitial fibrosis 【+ / -】Endocaridial thickening【+ / -】Intramural Artery
Two Forms of Coronary Artery AneurysmDilated AN Recanalized AN
Nomenclature and main histology
Initial LesionType I (initial) lesionisolated macrophage foam cells
Type II (fatty streak) lesionmainly intracellular lipid accumulationType III (intermediate) lesionType II changes & small extracellular lipid pools
Advanced LesionType IV (atheroma) lesionType II changes & core of extracellular lipidType V (fibroatheroma) lesionLipid core & fibrotic layer, or multiple lipid cores & fibrotic layers, or mainly calcific, or mainly fibroticType VI (complicated) lesionSurface defect, hematoma-hemorrhage, thrombusStary H C et al. Circulation 1995;92:1355-1374
A Definition of Advanced Types of Atherosclerotic Lesions and a Histological Classification of Atherosclerosis
Age Sex weight LMT-LAD LCX RCA
1 18 F 374g AN, Dil, Type III AN, Dil, Type II, AN, Dil,, Type IThromb occlusion
2 19 M AN, Dil, Type II non-AN, Type I AN, Dil, Type II, Thromb occlusion
3 19 M 370g AN, Dil, Type I non-AN, Type I AN, Recanal, Type I
4 20 F 320g AN、Recanal, Type I non-AN, Type I AN, Recanal, Type I
5 20 M AN, Recanal, Type II, non-AN, Type II AN, Dil, Type I
6 24 F 278g AN, Dil, Type VIThromb occlusion
AN, Dil, Type II, Thromb occlusion, AN, Recanal, Type I
7 25 M 446g non-AN, Type II non-AN, Type II non-AN, Type II
8 27 M 342g AN, Thromb occlusion, Type I, Thromb occlusion non-AN, Type I AN, Recanal, Type I
9 33 M 580g non-AN, Type I non-AN, Type II AN, Dil, Type II, Thromb occlusion
10 34 M 450g non-AN, Type V, Thromb occlusion non-AN, Type II non-AN, Type V
11 40 M 670g AN, Dil, Type V non-AN, Type II AN, Recanal, Type II
12 40 F 330g AN, Dil, Type I, Thromb occlusion AN, Dil, Type I AN, Dil, Type I
13 44 F 290g AN, Dil, Type I, Thromb occlusion non-AN , Type I AN, Dil, Recanal, Type II
14 45 M 527g AN, Dil, Type III, Thromb occlusion non-AN, Type II non-AN, Type II
15 50 M 333g AN, Dil, Type VI, Thromb occlusion non-AN, Type I AN, Recanal, Type II
16 57 M 345g AN, Dil,Type VI, Thromb occlusion
AN, Dil, Type VI, Thromb occlusion
AN, Dil, Type VI, Thromb occlusion
LMT-LADAN(+) (13pts)
Dilated(11)
Recanalized(2)An(-) (3pts)
LCXAN(+) (4pts) Dilated
(4)
An(-) (12pts)
RCAAN(+) (13pts)
Dilated(7)
Recanalized(6)An(-) (3pts)
Presence and Form of Aneurysm in each Coronary Artery Branch
Athero-sclerosis
Initial (Type I-III) Advanced (Type IV-VI)40 branches 8 branches
Coronary Artery Lesion
AN (+) AN(-) AN (+) AN(-)Dilated Recanalized Dilated Recanalized
16 8 16 6 0 2
Atherosclerosis and Coronary Artery Lesions
24yo: LAD, 40yo: LAD, 50yo: LAD, 57yo: LAD , LCX, RCA
Aneurysm AN(+) AN(-)13 1
Type of AN Dilated Recanalized13 0
Atheroscleroticlesion
Initial Advanced Initial Advanced Initial Advanced
8 5 0 0 0 1
Fourteen Coronary Artery Lesions with Thrombotic occlusion
18yo RCA, 19yo RCA, 24yo LCX, 27yo LAD, 33yo RCA, 40yo LAD, 44yo LAD, 45yo LAD
24yo LAD, 50yo LAD,57yo LAD, LCX, RCA
34yo LAD
24yo, female, LCX 57yo, male, LAD
2mm 2mm
Initial Lesion Advanced Lesion
Dilated Coronary Artery Aneurysm with Thrombotic Occlusion
40yo. Male, RCA44yo, female, RCA
Recanalized Coronary Artery Aneurysm -Initial Lesion -
25yo, male
LMT RCA
Non-aneurysmal Coronary Artery - Initial Lesion -
Histopathological studies were conducted on adult autopsy cases of patients who had either a history of KD or coronary artery aneurysms.
In regard to atherosclerosis, most coronary artery lesions were initial lesions (40/48 branches, 83%), while few were advanced lesions (8/48 branches, 17%).
Most advanced atherosclerotic lesions (75%) were observed to be in dilated aneurysms.
Thrombotic occlusion was found in 14/48 coronary artery branches. Almost all sites of thrombotic occlusion (13/14, 93%) were in a dilated aneurysm.
Advanced atherosclerotic lesion was confirmed in 5 of 13 branches of the dilated aneurysms with thrombotic occlusion. However, the remaining 8 branches of the thrombotic occlusions were seen in the initial lesion.
A pathological study of cases of adult sudden death due to coronary artery thrombosis (not be associated with KD) found that the 80% of cases of thrombotic occlusion were due to plaque rupture associated with advanced lesion, while only the 20% of cases were due to intimal erosion corresponding to initial lesion (Sato Y. Heart 91: 2005).
Our present findings indicate the possibility that, in adults as well as in children, a different mechanism of thrombus formation may be involved when there are remaining aneurysms compared with the cases of atherosclerosis.
Most cases of atherosclerosis with recanalized aneurysms and/or aneurysm-non-forming arteries were initial lesions, while thrombus formation was rare.
Does post-inflammatory arterial lesion become a risk factorof atherosclerosis?
- Investigation of sixteen autopsy cases over 18 years of age -
The dilated aneurysm is likely to become a risk factor for atherosclerosis and thrombotic occlusion. However, it must be kept in mind that thrombotic occlusion occur even in the initial atherosclerotic lesion in the dilated aneurysm.
Dilated AN (22)AdvancedAtherosclerosis (8)
Thrombotic Occlusion (14)
In case of recanalized aneurysm and aneurysm-non-forming artery, there are no evidence that both lesions have risk factors for atherosclerosis and for thrombotic occlusion.
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