<div>Title</div><div>Kawasaki disease: surgical treatment.</div><div>Authors and affiliations</div><div>Fabio Binhara Navarro, MD</div><div>Cardiothoracic Surgery</div><div>Hospital Pequeno Principe</div><div>Leonardo Mulinari, MD, PhD</div><div>Associate Professor of Surgery</div><div>Department of Surgery</div><div>University of Miami</div><div>Corresponding author</div><div>Leonardo Mulinari, MD, PhD</div><div>1611 NW 12th Ave</div><div>East Tower Suite 3016</div><div>Miami, FL, USA 33136-1015</div><div>Kawasaki disease: surgical treatment.</div><div>Abstract</div><div>Kawasaki Disease (KD) is a systemic vasculitis of small and medium
arteries, preferably affecting coronary arteries. It is one of the most
frequent causes of acquired heart disease in children. Despite being
comprehensively studied, its etiopathogenesis is not totally explained.
The surgical procedures usually become necessary during the late
follow-up and may be coronary artery bypass grafting, cardiac
defibrillator implantation with or without cardiac resynchronization
therapy, or cardiac transplantation.</div><div>Kawasaki Disease (KD) is a systemic vasculitis of small and medium
arteries, which preferably affects coronary arteries. It is one of the
most frequent causes of acquired heart disease in children. Despite
being comprehensively studied, its etiopathogenesis is not totally
explained. Probably genetic susceptibility allows an abnormal
immunological and inflammatory reaction triggered by a viral or
bacterial infection. Besides the fact of being described for more than
50 years, diagnostic confirmation is a clinical challenge. This happens
because its symptoms may be similar to bacterial or viral infections
and, mainly because there is no confirmatory laboratory test. The
diagnosis of the typical KD is made by the presence of fever for more
than five days in association with four of the five following signs and
symptoms: bilateral conjunctival edema or inflammation; changes in lips
or oral mucosa; polymorphic exanthema; cervical lymphadenopathy; edema,
erythema, or desquamation in hands or feet. Frequently, several
cardiovascular complications may occur, including valvulitis,
myocarditis, pericarditis, and even a circulatory shock (KD Shock
Syndrome). Coronary artery aneurysms (CAAs) and dilatation of them are
more often in the subacute phase, where up almost 20% of untreated
children may develop aneurysms.<sup>1-5</sup></div><div>The KD treatment is essentially medical, based on intravenous
immunoglobulins and aspirin in the acute phase. There are other
therapeutic options like corticosteroids, plasma exchange, TNF-a
inhibitors, cyclosporine, cyclophosphamide, and methotrexate. Patients
with giant coronary aneurysms ought to receive double platelets
anti-aggregation therapy and anticoagulation with warfarin or LWFH to
prevent coronary thrombosis. The earlier treatment, preferably within
the first 10 days of the disease, may assure a lower risk of CAA and
therapeutic failure. Regardless of the early and adequate therapy, 8%
of the children may exhibit cardiovascular complications, 6% with
coronary dilatation, 1% with CAA, and 0,13% with giant CAA. The
long-term management begins 4 to 6 weeks after the fever onset when the
acute symptoms have already disappeared and the coronary involvement
reached the maximum extent. At this moment, the treatment objective is
to prevent coronary thrombosis and myocardial ischemia. Even after the
resolution of the inflammatory process and luminal diameter regression,
the coronary artery wall structure and function may be abnormal, leading
to progressive stenosis and occlusion over time. The acute coronary
occlusion treatment is done with thrombolytic agents and, if the patient
has adequate size can be done percutaneously. Patients with moderate
coronary aneurysms (≥6mm) present a higher risk of developing coronary
progressive occlusion or Acute Coronary Syndrome. However, this
propensity increases exponentially in patients with large aneurysms
(luminal diameter Z value ≥ 10 or ≥ 8mm), demanding a close follow-up.<sup>4, 6-9</sup></div><div>The surgical procedures usually become necessary during the late
follow-up. As Wang, Z et al have described in this issue of the JCS,
surgical interventions may be necessary for treating myocardial ischemia
when there is no possibility of percutaneous coronary intervention
(PCI). When the patient has cardiomyopathy, due to coronary occlusion
and myocardial ischemia or secondary to the initial myocardial insult
during the acute KD, cardiac defibrillator implantation with or without
cardiac resynchronization therapy, or cardiac transplantation may be
necessary.</div><div>When coronary artery bypass grafting (CABG) is necessary, the precise
comprehension of the myocardial ischemia mechanism and myocardial
viability evaluation is very important, since it can be due to coronary
stenosis or secondary to recurrent thrombosis of coronary aneurysms. In
the discussed article, this mechanism is not precisely described, but,
in clinical practice, it is mandatory, requiring complementary exams
like myocardial scintigraphy, coronary tomography, magnetic resonance,
and/or coronary angiography, preferable associated with IVUS and
FFR.<sup>7,10</sup></div><div>The CABG has excellent results in KD and, likewise adults, the<i>in-situ</i> artery grafts have better long-term patency in children.
In addition, these arterial grafts have a growing potential that may
follow the somatic child’s growth, which does not happen with saphenous
vein grafts. Hence, the elective choice of saphenous vein grafts, even
using the no-touch technic, needs more scientific validation, mainly in
smaller children.<sup>7,11</sup></div><div>Albeit not found in the discussed manuscript, valvular lesions may occur
and require surgical alleviation. During the acute phase, in rare
situations (1,5% of cases), mild or moderate mitral insufficiency can
happen, secondary to valvulitis or sub-valvar apparatus damage.
Generally, this insufficiency diminishes after the resolution of the
inflammatory state. Likewise, ascending aorta inflammation and
dilatation of the aortic root have also been described, leading to
secondary aortic insufficiency. Mitral or aortic valvar repair or
replacement have already been reported, despite being very
uncommon.<sup>7, 12, 13</sup></div><div>1. Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid
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