Cardiology in the Young (2011), 21, 74–82 r Cambridge University Press, 2010

doi:10.1017/S1047951110001502

Original Article

Acute coronary syndrome in adult patients with

coronary artery lesions caused by Kawasaki disease:
review of case reports
Etsuko Tsuda, Tadaaki Abe, Wataru Tamaki

Department of Pediatrics, National Cardiovascular Center, Osaka, Japan

Abstract Information about acute coronary syndrome caused by Kawasaki disease-related coronary artery lesions
in adults is sketchy. We reviewed the clinical features of 50 adult patients who had an acute coronary syndrome
caused by coronary artery lesions due to Kawasaki disease or probable Kawasaki disease from 1980 to 2008. Of the
50 patients, 43 (90%) were male and seven were female (10%). Their ages at the onset of acute coronary syndrome
ranged from 18 to 69 years, with a median of 28 years. The culprit lesion in 43 patients was thrombotic occlusion
of an aneurysm, and 40 patients had giant aneurysms. In the three patients in whom no aneurysms were seen in
coronary angiograms performed at the time of acute myocardial infarction, either giant aneurysms or aneurysms had
been visualised in childhood. The initial treatment of acute coronary syndrome was as follows: intracoronary
thrombolysis, 11; primary percutaneous coronary intervention, 9; emergency coronary artery bypass grafting, 3; and
medication, 26. Elective coronary artery bypass grafting was performed in 15 patients. Three patients (6%) died.
Of the 27 patients with additional coronary risk factors, 20 were smokers. Giant aneurysms due to Kawasaki disease
continued to cause acute coronary syndrome in adult life with onset at a younger age than typifies that due to
atherosclerosis in the general population, especially in male population rather than female population. Even when
giant aneurysms regressed after the acute phase, a few patients still developed acute coronary syndrome in adult life.
Smoking appears to be the most prominent additional risk factor.

Keywords: Kawasaki disease; giant aneurysm; acute coronary syndrome; coronary artery calcification; smoking

Received: 7 October 2009; Accepted: 12 September 2010; First published online: 12 November 2010

about the more common acute coronary syndrome

K
AWASAKI DISEASE IS AN ACUTE VASCULITIS OF
unknown origin, which causes coronary
artery lesions leading to ischaemic cardiac
disease in about 1–2% of affected patients.1
Kawasaki disease has been occurring worldwide
for the past four decades. Acute coronary syndrome
complicating coronary artery disease is a major
determinant of prognosis for such patients and
should be prevented if possible.2 However, informa-
tion about acute coronary syndrome caused by
Kawasaki disease-related coronary artery lesions in
adults is limited compared with our knowledge
Correspondence to: E. Tsuda, MD, Department of Pediatrics, National
Cardiovascular Center, 5-7-1 Fujishirodai, Suita-shi, Osaka, 565-8565, Japan.
Tel: 81 6 6833 5012; Fax: 81 66872 7486; E-mail: etsuda@hsp.ncvc.go.jp
due to atherosclerosis.3 However, the number of
adults with coronary artery lesions caused by
Kawasaki disease is gradually increasing, and they
are surviving to an older age. Consequently, a better
understanding of the natural history of coronary
artery lesions and the clinical features of acute
coronary syndromes in this population should not
only help to anticipate future cardiac events but also
improve their management when they occur.
Patients and methods
We reviewed the clinical features of adult patients
with acute coronary syndrome caused by Kawasaki
disease-related coronary artery lesions, and included
Vol. 21, No. 1 Tsuda et al: Acute coronary syndrome after Kawasaki disease
a few patients with presumed Kawasaki disease. We
also included acute coronary syndrome due to coronary
artery lesions characteristic of Kawasaki disease, even
if a history of Kawasaki disease was undocumented.
Coronary artery lesions characteristic of Kawasaki
disease include giant aneurysms with a diameter
exceeding 8 millimetres; coronary artery calcification;
segmental stenosis, which implies recanalisation
after thrombotic occlusion; and aneurysms involv-
ing the left coronary artery bifurcation.1,4 Further-
more, Kawasaki disease-related coronary artery lesions
usually involve the proximal segments of the major
branches and tend to be localised, both the affected
areas and apparently normal areas coexisting in the
same patient. Acute coronary syndrome implies acute
myocardial infarction or unstable angina, and includes
sudden deaths caused by acute myocardial infarction.
Patients with stable angina, syncope, and sudden
deaths due to ventricular arrhythmia attributed to
previous myocardial infarction were excluded from
this study.
We identified 43 patients who met the above
criteria and who were reported in the literature from
37 references between 1980 and 2008 (Table 1). The
number of references per decade was: 1980–1989: 7;
1990–1999: 15; and 2000–2008: 15. From 1985 to
2003, three patients were referred to our hospital,
and from 1993 to 2005, four patients were treated
for acute coronary syndrome in our hospital. Of
these 50 patients, 43 (90%) were male and seven
(10%) were female. Thirty-five patients (70%) were
Japanese. Coronary artery lesions were previously
confirmed by selective coronary angiograms in 48 of
the 50 patients, and in the remaining two patients
with a history of Kawasaki disease, their coronary
artery lesions were described in the reference.5
Whether the patients had coronary artery risk factors
was recorded in 34 of the references examined and we
analysed risk factors in 43 patients. Coronary arterial
risk factors in this study included obesity, hyperten-
sion, hyperlipidaemia, diabetes mellitus, and smoking.
The clinical features of acute coronary syndrome in
these patients were analysed with the following results.
Results
Age and clinical features at the onset of acute
coronary syndrome
Age at the onset of acute coronary syndrome ranged
from 18 to 69 years with a median of 28 years.
The number of patients by age at the time of acute
coronary syndrome is shown in Figure 1. Of the
patients, 45 (90%) experienced acute coronary
syndrome when less than 40 years old. The time
of onset of acute coronary syndrome was recorded in
75
25 of the 50 patients, and was from midnight to
early morning in 19 patients (76%). Anti-platelet
agents were administered in 4 of 50 patients (8%)
before the onset of acute coronary syndrome.
Of the acute coronary syndrome events, 45 (90%)
were acute myocardial infarction, and five (10%)
were unstable angina (Table 1). Among the 45
patients, two had progressed from unstable angina
to acute myocardial infarction. Symptoms associated
with acute coronary syndrome included syncope in
four patients and chest pain in 46 patients. In the
four patients who had syncope, either ventricular
fibrillation or ventricular tachycardia was detected
and successfully converted. Premonitory symp-
toms occurred in only five patients (10%) before
acute coronary syndrome. On two occasions, two
patients experienced ST-elevated myocardial infarc-
tion (patients 9, 26). There were 47 patients (94%)
who survived their acute coronary syndrome, and
three patients (6%) died. A 26-year-old male patient
with a slightly dilated left anterior descending
artery died suddenly after complaining of chest
pain, and autopsy revealed an acute anteroseptal
myocardial infarction caused by the thrombotic
occlusion of the left anterior-descending artery.
Despite there being no significant localised stenosis,
thickening and calcification of the proximal portion
of the left anterior descending artery was present
(patient 50). A 19-year-old male patient with
cardiac failure due to acute myocardial infarction
died of recurrent myocardial infarction 26 days after
initial onset. Extreme luminal narrowing with
severe intimal thickening was observed in the
proximal portion of both coronary arteries at the
autopsy (patient 6). A 69-year-old female patient
died of pneumonia, after an emergency coronary
artery bypass grafting and repair of an acquired
ventricular septal defect complicating her acute
myocardial infarction (patient 29).
There were 27 patients (54%) with an anteroseptal
myocardial infarction caused by left anterior descend-
ing artery occlusion, while an inferior myocardial
infarction due to an occluded right coronary artery
was found in 16 patients (32%; Table 1). There were
2 patients (4%) with posteroinferior myocardial
infarction caused by occlusion of the left circumflex.
The culprit lesion in 39 patients was thrombotic
occlusion of an aneurysm, which was gigantic in
36 patients. There were three patients, in whom no
aneurysm was seen in the coronary angiograms at the
time of acute myocardial infarction, and who had
previously documented giant aneurysms or aneur-
ysms in childhood, which had regressed (patients 19,
44, and 50). In the remaining three patients, the
existence of an aneurysm in the acute phase was
unknown (patients 6, 43, and 49). The culprit lesions
 76
Table 1. Clinical characteristics in 50 patients with acute coronary syndrome.

Characteristic coronary artery lesions

Diagnosis Author or
Patients Age Sex (location) Treatment GA Cal LMT SS Past history Risk factor Year journal*** institution

1 38 M AMI (INF) med 1 HL 1980 Letac B15

2 45 M AMI (ANT) med 1 S 1980 Letac B15
3 28 M AMI (ANT) med 1 1 1984 Oliveria DBG16
1988
4* 30 M AMI (INF) med-CABG 1 S SHINZO Mukae S
5 30 M AMI (ANT) med 1 1 1 8y 1988 Brecker SJD17
KD** 1989 Patho clinic
6* 19 M AMI (INF) med 1 S Med Watanabe H
7* 22 M AMI (ANT) med-CABG 1 1 1 1989 NCVC
8* 32 M AMI (ANT) med AN 1989*** Asada S
9* 36 M AMI (INF) med AN 1 1989*** Asada S
10* 24 M AMI (ANT) ICT-POBA 1 1 1 1y KD** S 1989*** Hayashi Y

Cardiology in the Young

11* 37 F AMI (ANT) ICT 1 2y UF 1991 Myler RK18
12* 37 M AMI (ANT) med 1 6y KD** 1992 Kato et al19
13 28 M AMI (ANT) ICT-CABG 1 HT HL 1992 Morita et al20
1993
14* 19 M AMI (ANT) ICT-CABG 1 1 1 9y KD SHINZO Takeuchi H
15* 54 M UA-AMI (ANT) med-CABG 1 1 6y KD** 1993*** Imamura K
16 19 M AMI (PI) med 1 1 1 13y S 1994 Pongratz et al21
3y 1994
17* 18 M AMI (INF) ICT 1 KD** Prog Med Baden M
9y 1994
18* 29 M AMI (ANT) ICT 1 1 1 KD** S Prog Med Baden M
9y
19 26 M AMI (ANT) med-CABG 1 1 1 KD** 1994 Albat et al22
5y
20* 31 M AMI (INF) ICT AN 1 KD** S 1994 Pharma Medica Wada T
6y
21* 37 F AMI (INF) med 1 1 1 KD** 1996 Satoh et al23
S DM HL 1996 Jpn J Thora
22* 44 M AMI (ANT) med-CABG 1 1 6y UF OB Surg Imazeki T
23* 19 M UA em CABG 1 1 14y KD** 1997 NCVC
24* 24 M AMI (INF) ICT POBA 1 1 6y KD OB 1997 NCVC
25 32 M AMI (INF) med-CABG 1 S 1997 Shapira et al24
26 37 M AMI (INF) med 1 1 3y KD S 1998 Habon et al25

February 2011
3y 1999
27* 22 M AMI (AMI) med-CABG 1 1 1 KD** SHINZO Daimon M
1999
28* 24 F UA (INF) med 1 1 SHINZO Daimon M
AMI (ANT)
 Vol. 21, No. 1
Table 1. Continued

Characteristic coronary artery lesions

Diagnosis Author or
Patients Age Sex (location) Treatment GA Cal LMT SS Past history Risk factor Year journal*** institution

29* 69 F Perforation em CABG 1 1 1 1y OB 1999 Okayama hospital

30 18 M AMI (ANT) med 1 1 1 KD** 2000 Lier et al26
UA-AMI
31* 33 M (ANT) med-CABG 1 1 1 4y UF S 2000 Kudou M
4y 2003
32* 19 M AMI (INF) POBA-PTCRA 1 KD** Jap J Interv Cardiol Sugiura T
33* 26 M AMI (INF) POBA-CABG 1 OB 2003 NCVC
34* 27 M AMI (INF) ASP DCA 1y KD 2003 Negoro et al27
35* 32 M UA (ANT) DCA 1 1 1 1 2y KD S 2003 Negoro et al27
Mirza et al28

Tsuda et al: Acute coronary syndrome after Kawasaki disease

36 19 M AMI (ANT) em CABG 1 1 1 2y KD 2004
37 25 M AMI (ANT) med-CABG 1 1 1 S 2004 Su et al29
3y3m 2004
38* 25 M AMI (INF) ICT 1 1 KD Prog Med Shinohara T
39* 33 F UA (INF) ASP POBA 1 1 6y KD S OB 2004 NCVC
40* 39 M AMI (INF) ICT-STENT 1 1 KD S 2004 Parisi Q5
41* 45 M AMI (ANT) med-CABG 1 1 1 UF HT 2004 Parisi et al5
42* 20 M AMI (ANT) POBA ICT 1 S 2005 Shiraishi J et al30
43* 26 M AMI (ANT) POBA 1 3y KD S 2005 Shiraishi J et al30
44* 26 M AMI (ANT) ICT 1 8m KD S 2006 NCVC7
2006
45* 32 F AMI (ANT) med 1 1 1 OB J Cardiovasc Cathe Motohiro M
2006
46* 33 M AMI (PI) ASP 1 S HL SHINZO Kadotani M
47* 30 M UA (ANT) med-CABG 1 1 1 3y KD 2006 NCVC
4y 2007
48* 27 M AMI (ANT) ASP STENT 1 1 KD** S SHINZO Watanabe T
2008
49* 23 M AMI (ANT) med-CABG 1 J Jpn coron assoc Nakamura T
50* 26 M AMI (ANT) Sudden death 1 1y, 3y KD 2008 Prog Med Fujiwara Y
AMI 5 acute myocardial infarction; AN 5 aneurysm; ANT 5anterior; ASP 5 aspiration; CABG 5 coronary artery bypass grafting; Cal 5 calcification; DCA 5 directional coronary atherectomy;
DM 5 diabetes mellitus; em CABG 5 emergency CABG; GA 5 giant aneurysm; HL 5 hyperlipidaemia; HT 5 hypertension; ICT 5 intracoronary thrombolysis; KD 5 Kawasaki disease;
med 5 medication; NCVC 5 National Cardiovascular Center in Japan; OB 5 obesity; PI 5 posteroinferior; POBA 5 percutaneous balloon angioplasty; PTCRA 5 percutaneous transluminal coronary
rotational ablation; S 5 smoking; SS 5 segment stenosis; UA 5 unstable angina; UF 5 unknown fever; UN 5 unknown
*Japanese, **patient with UF and major symptoms (presumed KD), ***Journals written in Japanese

77
78 Cardiology in the Young
Figure 1.
Age distribution at the time of acute coronary syndrome.
of unstable angina were as follows: the left anterior
descending artery, two patients; the right coronary
artery, 2 patients; and the left main trunk, 1 patient.
There were four, out of five, patients with giant
aneurysms.
Treatment of acute coronary syndrome
To support left ventricular function, intra-aortic
balloon pumping was used in four patients, and
percutaneous cardiopulmonary support was provided
in one patient. Ventricular tachycardia after acute
myocardial infarction occurred in two patients and
transient complete atrioventricular block in one
patient. Acute coronary syndrome was treated by
intracoronary thrombolysis in 11 patients; percuta-
neous balloon angioplasty was added in two patients,
and a stent was implanted in one patient. The
procedure was successful in nine patients, and was
followed by elective coronary artery bypass grafting in
two of the 11 patients (Fig 2). Primary percutaneous
coronary intervention was successful in nine patients.
Thrombus aspiration was performed in four patients,
and percutaneous balloon angioplasty, directional
coronary atherectomy, and stent implantation was
added in one patient each; percutaneous balloon
angioplasty and directional coronary atherectomy were
performed in three patients and one patient, respec-
tively; one patient underwent intracoronary thrombo-
lysis immediately after successful percutaneous balloon
angioplasty. Coronary revascularisation was successful
in all nine patients with primary percutaneous
coronary intervention. Elective coronary artery bypass
grafting and elective percutaneous transluminal
coronary rotational ablation were performed in one
patient for each procedure after primary percutaneous
coronary intervention. There were three patients who
February 2011
Figure 2.
Treatment and outcome for acute coronary syndrome (ICT 5
intracoronary thrombolysis; PCI 5 percutaneous coronary inter-
vention; CABG 5 coronary artery bypass grafting).
underwent emergency coronary artery bypass graft-
ing; 26 patients took medication immediately after
acute coronary syndrome, and 12 patients under-
went elective coronary artery bypass grafting.
Left ventricular ejection fraction
Left ventricular ejection fraction was measured in
24 patients, acute myocardial infarction in 20 patients
and unstable angina in four patients. Among the
24 patients, 11 had undergone either primary per-
cutaneous coronary intervention or intracoronary
thrombolysis, and the procedure had been successful
in nine. There were 11 patients who underwent
elective coronary artery bypass grafting, and three
patients who had not undergone coronary revascu-
larisation. In 17 patients (71%), left ventricular
ejection fraction was less than 50%. Among the
seven patients who had a left ventricular ejection
fraction more than or equal to 50%, three were
diagnosed with unstable angina.
Birth year and past history
The year of birth was available either in the references
or our medical records for 37 patients (75%). The
number of births per decade were: 1920–1929,
1; 1930–1939, 3; 1940–1949, 4; 1950–1959, 6;
1960–1969, 8; 1970–1979, 14; and 1980–1989, 1
(Fig 3). Kawasaki disease was diagnosed at the time
of the acute illness in 13 patients who were born in
the 1960s and 1970s. There were 14 patients with
an undiagnosed acute febrile illness with the major
symptoms of Kawasaki disease. Otherwise, a history
of unknown fever in childhood was recorded in
four patients, and it was unknown in the remaining
19 patients.
Vol. 21, No. 1 Tsuda et al: Acute coronary syndrome after Kawasaki disease
Figure 3.
Birth year and past history in patients with acute coronary
syndrome (KD 5 Kawasaki disease).
In the 13 patients with a history of Kawasaki disease,
the age at the onset of Kawasaki disease ranged from
8 months to 13 years, with a median of median 3 years,
and the interval from the onset of Kawasaki disease to
the onset of acute coronary syndrome was from 10 to 34
years, with a median of 25 years. Coronary artery lesions
caused by Kawasaki disease had not been diagnosed in
five of the 13 patients until an acute coronary syndrome
developed, although the diagnosis of acute Kawasaki
disease had been made. The three patients who were
previously diagnosed as having coronary artery lesions
developed acute coronary syndrome after discontinuing
medication. There were two patients who had acute
myocardial infarction later, despite their being diag-
nosed as having apparently normal coronary arteries on
an earlier coronary angiogram. In the 14 patients with
unknown fever with the major symptoms of Kawasaki
disease, the age at the onset of febrile illness ranged
from 1 to 9 years, with a median of 4 years, and the
interval from the onset of febrile illness to the onset of
acute coronary syndrome was from 5 to 48 years, with a
median of 20 years. Of the 14 patients, three had been
diagnosed as rheumatic fever. The diagnosis of mumps
and scarlet fever was made in one patient for each
disease. However, on retrospective review, the unknown
febrile illness was strongly suspected to have been acute
Kawasaki disease.
Incidence of characteristic coronary artery lesions
caused by Kawasaki disease
In all, three-vessel disease affected 24 patients, and
two-vessel and one-vessel disease involved 17 and 9
patients, respectively. As to the characteristic coronary
artery lesions caused by Kawasaki disease, 21 patients
79
(43%) had giant aneurysms with coronary calcifica-
tion; 19 patients (38%) had giant aneurysms; four
patients (8%) had coronary artery calcification; and
three patients (4%) had an aneurysm. There were
three patients with neither an aneurysm nor coronary
artery calcification. However, in one of the three
patients, prior existence of aneurysms was confirmed
from previous coronary angiograms. The coronary
artery lesions are summarised in Table 1. Segmental
stenosis, which implies recanalisation after complete
occlusion of a major coronary artery, was found in
10 patients (20%), and giant aneurysms at the bifur-
cation of the left coronary artery were present in
21 patients (42%).
Calcification at the cardiac area on a chest X-ray
photogram was detected in five patients. Severe
intimal thickening in the coronary arterial wall
was found in all eight patients who underwent
intravascular ultrasound, and a heterogeneous echo
indicating atherosclerotic plaque was detected in
two patients with coronary risk factors (patients 35
and 40). In the specimens obtained by directional
coronary atherectomy or aspiration, microscopy
revealed cholesterol crystals and macrophages in
one of these two patients (patient 35). No athero-
sclerosis was found at operation in one patient
(patient 11). At autopsy, both the 19-year-old and
26-year-old male patients had severe intimal
thickening of the proximal portion of the major
branches, with no apparent atherosclerotic change.
Coincident risk factors for coronary artery disease
Coronary risk factors were present in 27 (63%) out
of 43 patients. Risk factors per patient were one
factor in 24, two in 3, and four in 1. Smokers
constituted 20 patients (47%). Other risk factors
were as follows: obesity, 6; hyperlipidaemia, 3;
hypertension, 2; and diabetes, 1. The most common
risk factor was smoking, and the number of smokers
by age at acute coronary syndrome is shown in
Figure 4. There were 18 (78%) smokers among the
23 patients with coronary risk factors and they were
less than 40 years old.
Discussion
Giant calcified aneurysms involving the proximal
portion of the major branches were the most
common culprit lesions found, and in most cases
thrombus formation within the aneurysm precipi-
tated the acute coronary syndrome. Despite the
incidence of thrombotic occlusion in giant aneur-
ysms in the late period being low compared with
patients with giant aneurysms within the first year
after the acute Kawasaki disease, it emphasises that
80 Cardiology in the Young
Figure 4.
Coronary risk factors by age at acute coronary syndrome.
persistent giant aneurysms remain an important risk
factor for acute coronary syndrome many years after
the acute febrile episode.6 The national biennial
survey in Japan reported that about one-third of
patients with giant aneurysms after the acute illness
were female. In this study, female patients with
acute coronary syndrome constituted only one-tenth
of the cohort. For people under 50 years of age, there is
usually a gender bias towards male population in the
incidence of coronary artery disease. However, the
gender difference in the incidence of acute coronary
syndrome in this population may be bigger.
Some patients developed thrombotic occlusion at
the sites where aneurysms had pre-existed, but had
regressed.7 Even if giant aneurysms regress, they remain
a risk for acute coronary syndrome under certain
conditions, for example, the addition of other coronary
risk factors. Coronary angiograms after revascularisation
revealed apparently normal arteries in cases with
regressed aneurysms. A detailed evaluation of coronary
artery lesions caused by Kawasaki disease should be
performed early in young acute coronary syndrome
patients without apparent coronary risk factors, and
may require multi-row detector computed tomography
and intravascular ultrasound to assess intimal thicken-
ing and atherosclerotic plaque. However, the differential
diagnosis of coronary artery lesions caused by Kawasaki
disease versus those due to atherosclerosis with ageing
may be difficult in some adult cases.
Aneurysms caused by Kawasaki disease have
persisted long into adulthood after the acute illness
in childhood with secondary vessel wall thicken-
ing in most cases.4 Coronary artery calcification
indicates severe intimal thickening of the coronary
wall. Coronary aneurysms larger than a 6-millimetre
February 2011
diameter in the acute phase are frequently calcified
after more than 10 years.8 Thus, calcification can be
considered as a marker of coronary artery lesions
caused by acute Kawasaki disease experienced many
years earlier, especially in patients with apparently
normal coronary arteries on angiography. Even when
the aneurysms have regressed in coronary angio-
grams, calcification indicates involvement of the
coronary artery wall in the acute phase of Kawasaki
disease.
The incidence of large aneurysms is 1.4–4.9% in
the general adult population,9,10 and is most
commonly due to atherosclerosis. Destruction of
the muscular media weakens the vessel wall,
resulting in subsequent focal dilatation. Aneurysms
also complicate atherosclerosis, and therefore the
finding of non-calcified giant aneurysms does not
necessarily imply a Kawasaki disease aetiology;
furthermore, coronary artery calcification increases
with ageing.11 However, the location of coronary
artery calcification and aneurysms helps to differ-
entiate between Kawasaki and atherosclerotic
aetiology.12 In Kawasaki disease, calcification is
usually limited to the site of pre-existing aneur-
ysms, while in atherosclerosis, it is not co-located
with aneurysms. Further, lesions due to athero-
sclerosis are diffuse, while they are usually localised
in Kawasaki disease as they reflect the degree of
acute vasculitis. Coronary artery lesions caused by
Kawasaki disease rarely involve the small branches
and peripheral coronary arteries.
The treatment for acute coronary syndrome has
improved remarkably over the past three decades;
thanks to thrombolytic therapy and improved
percutaneous coronary intervention. Early revascu-
larisation after acute myocardial infarction mini-
mises the infarct area, and it was applied to some
patients in this population, but simple aspiration or
thrombolytic therapy was not always successful
because of the large volume of thrombus in the
giant aneurysm. In such cases, percutaneous balloon
angioplasty was often added.
Kawasaki disease was not always recognised
especially in the 1960s and 1970s when many
physicians were unfamiliar with its features. The
diagnosis of Kawasaki disease is clinical and is based
on the major characteristic clinical features of the
acute phase, and therefore there are undoubtedly
many asymptomatic adult patients with coronary
arterial lesions caused by Kawasaki disease who
remain undiagnosed, forming a hidden cohort with
this disease.4 Even when acute Kawasaki disease was
recognised, the diagnosis of complicating coronary
artery lesions was more difficult in the sixties and
seventies. Only recently have technical develop-
ments allowed a detailed examination of coronary
Vol. 21, No. 1 Tsuda et al: Acute coronary syndrome after Kawasaki disease
artery morphology. Symptoms are rare in this
population until the onset of acute coronary syndrome;
consequently, the presence of coronary artery disease
was unsuspected in most of the patients in this study
until the actual episode and preventive antithrombotic
therapy was not considered. Worth stressing is the
occurrence of acute coronary syndrome in a few
patients in whom aneurysms had apparently regressed
as demonstrated by coronary angiograms performed
before the episode. Medication had been stopped in
them, because the giant aneurysms had regressed.
Whether or not to continue antithrombotic therapy
in patients with regressed giant aneurysms is an
important problem to resolve in the future.
In the general adult population, less than 10%
of patients are under 50 years of age when they
develop acute coronary syndrome.13 Acute coronary
syndrome in adults with coronary artery lesions due
to Kawasaki disease occurs at a younger age than
acute coronary syndrome in the general population.
The incidence of coronary risk factors in this
population was expected to be low compared with
that in the usual atherosclerotic acute coronary
syndrome due to ageing. In the Kawasaki disease
patients, the specific nature of the Kawasaki disease-
related lesions seems to strongly influence the onset
of acute coronary syndrome rather than the athero-
sclerotic factors. However, smoking was found in
about half of the patients in this study, and was the
most prominent risk factor. Smoking may be closely
related to the onset of acute coronary syndrome in
this population as in general adult studies pre-
viously reported,14 and it is clearly contraindicated
in these patients.
Long-term careful follow-up is needed in patients
with coronary artery lesions caused by Kawasaki
disease, because treatment becomes more essential as
they age and acquire new coronary risk factors.
However, as most patients remain asymptomatic
until the onset of acute coronary syndrome, it has
been difficult to justify continued medication in
some patients in the absence of a real understanding
of the long-term disease profile from childhood to
adulthood. Patients tend to drop out of follow-up as
they reach adulthood; furthermore, coronary artery
lesions caused by Kawasaki disease are not always
familiar to internists because the acute illness occurs
in childhood and the affected population is small.
The true adult population with coronary artery
lesions caused by Kawasaki disease is difficult to
estimate. This study focused on the clinical features
of acute coronary syndrome in this population.
More needs to be learned about the natural history,
pre-acute coronary syndrome recognition and the
prevention of acute coronary syndrome in this
uncommon but life-threatening condition.
81
Conclusion
Giant aneurysms due to Kawasaki disease eventually
cause acute coronary syndrome, the onset being at
a younger age than is typical for the much more
common atherosclerotic acute coronary syndrome.
This seems to be especially true in male. Even if
the aneurysms regress, the risk of acute coronary
syndrome remains in adult life. Smoking was a
prominent additional risk factor.
Acknowledgements
We thank Professor Peter Olley for his kind English
language consultation.
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