Coronary artery aneurysm occurs in approximately
20% of untreated patients with KD, whereas it
accounts for only 4% to 8% of children who receive
intravenous gamma globulin.[
4] Currently, early
high-dose intravenous gamma globulin is accepted
as the standard initial therapy. Late intravenous
gamma globulin administration may also decrease
inflammation, but may not be able to prevent coronary
artery lesions.[
5,
6] In the presence of clinical suspicion,
persisting fever less than five days may be considered
to be a positive diagnostic criterion and intravenous
gamma globulin can be employed.[
7] Chen et al.[
8]
analyzed nine studies investigating the progression of
coronary involvement in KD and concluded that the
combination of a corticosteroid with the conventional
regimen of intravenous gamma globulin as the initial
treatment strategy might reduce the risk of coronary
abnormality.[
8,
9] Currently, TNF alpha antagonists are
used, particularly in patients with intravenous gamma
globulin resistance.[
10] Therefore, our patient was considered inappropriate for TNF alpha antagonist
therapy due to late presentation.
Kawasaki disease generally affects proximal
segments of the coronaries. In our patient, the left
anterior descending artery was diffusely diseased
and the circumflex artery was segmentally involved.
Our patient received neither immunoglobulin nor
steroid therapy previously and experienced prolonged
periods of fever several times. The association of
prolonged fever with coronary artery lesions has
been well established.[11] Frequent cardiac imaging in
fever episodes may be helpful, since children cannot
distinguish ischemic symptoms.
Although coronary aneurysms due to KD may
be asymptomatic, myocardial infarction and rupture
are life-threatening complications which are rarely
reported in the literature. Aneurysms may cause
thrombosis, distal embolization, calcification, coronary
stenosis, myocardial ischemia, infarction, rupture,
cardiac tamponade and even death. Akagi et al.[12]
reported a 1.5% incidence of myocardial infarction in
patients with a giant coronary artery aneurysm during
a four-year follow-up period. Coronary aneurysms do
not tend to rupture except in acute illness. Ruptured
aneurysms have been noted as case reports and, to
the best of our knowledge, there is no satisfactory
study investigating the incidence and risk factors for
aneurysm rupture. The selection of the ideal surgical
technique is still controversial. In symptomatic
patients, CABG with or without aneurysmectomy
is the preferred approach. Aneurysmectomy can be performed in selected cases, however, it may cause
difficulties in revascularization and the protection of
branches. On the other hand, ligation of the aneurysm
and distal bypass is a controversial alternative due to
the risk of subsequent graft failure.[13] Coronary artery
bypass graft surgery with internal thoracic artery
graft is the gold standard in patients suffering from
myocardial ischemia.[13] The internal thoracic artery
is the preferred graft with a high long-term patency
rate.[13] In our patient, we utilized bilateral internal
thoracic artery grafts for revascularization.
In conclusion, giant coronary aneurysm is a very
rare and life-threatening complication of KD. Early
diagnosis and aggressive treatment may prevent
aneurysm formation. Coronary artery bypass grafting
is a safe and preferred approach with encouraging
long-term results in patients suffering from myocardial
ischemia.
Declaration of conflicting interests
The authors declared no conflicts of interest with
respect to the authorship and/or publication of this
article.
Funding
The authors received no financial support for the
research and/or authorship of this article.
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