• Users Online: 152
  • Print this page
  • Email this page

Table of Contents
Year : 2021  |  Volume : 11  |  Issue : 2  |  Page : 86-88

Coronary artery complication in kawasaki disease and management option

1 Department of Pediatric Cardiology, NH RTIICS, Kolkata, West Bengal, India
2 Department of Pediatric Cardiology, NSH, Howrah, West Bengal, India

Date of Submission12-Apr-2020
Date of Decision08-May-2020
Date of Acceptance17-May-2020
Date of Web Publication03-May-2021

Correspondence Address:
Dr. Mahua Roy
931, Jawpur Road, Kolkata - 700 074, West Bengal
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JICC.JICC_23_20

Rights and Permissions

Kawasaki disease (KD) is a vasculitis of medium-sized arteries of which coronary artery involvement is of particular concern. Here, we are presenting three cases of KD with coronary artery aneurysm. One patient was treated with oral antiplatelet and anticoagulant, another one with impending coronary artery occlusion was managed by infusion of thrombolytic medication and last one, neglected KD patient needed coronary artery bypass grafting.

Keywords: Coronary artery aneurysm, Coronary artery bypass grafting, Kawasaki disease, Thrombolytic medication

How to cite this article:
Roy M, Gangopadhyay D, Chattopadhyay A. Coronary artery complication in kawasaki disease and management option. J Indian coll cardiol 2021;11:86-8

How to cite this URL:
Roy M, Gangopadhyay D, Chattopadhyay A. Coronary artery complication in kawasaki disease and management option. J Indian coll cardiol [serial online] 2021 [cited 2021 Sep 18];11:86-8. Available from:

  Introduction Top

Kawasaki disease (KD) is a vasculitis of medium-sized arteries of which coronary artery involvement is of particular concern. Here, we are presenting three cases of complicated KD. Two of them were treated in acute phase with Intravenous immunoglobulin (IVIG) along with anti-platelet and/or thrombolytic medication, another one neglected KD patient needed coronary artery bypass grafting (CABG).

  Case Reports Top

Case 1

A 4 months old boy was reviewed for a febrile illness with oral mucosal redness. He was diagnosed as atypical KD following echocardiogram which demonstrated multiple small and moderate aneurysms in the proximal right coronary artery and left anterior descending (LAD) artery [Figure 1]. The disease responded favorably to single dose of intravenous immunoglobulin and was discharged on oral aspirin and clopidogrel.
Figure 1: 2D Echocardiography showing dilated right coronary artery and left main coronary artery

Click here to view

Case 2

A 29-day-old boy was referred as a case of neonatal sepsis not responding to antibiotics. Clinically, he had erythematous rash and persistent high-grade fever for more than a week. Blood investigations revealed elevated total white cell count, C-reactive protein (CRP), and thrombocytosis. Multiple blood cultures were negative. Echocardiography showed a giant aneurysm in the left main coronary artery (LMCA) and another one in LAD and large thrombus almost occluded both arteries [Figure 2]. He was treated with IVIG however, due to persistent fever and inflammatory markers even 48 h after completion of IVIG, 2nd dose was also given. Streptokinase (STK) infusion was also started but thrombus remained same after 24 h of infusion. Hence, infusion alteplase (recombinant tissue plasminogen activator [tPA]) and tirofiban (antiplatelet) were administered. With this treatment, fever subsided and thrombus were reduced in size thus establishing blood flow across the left coronary system. The baby showed significant clinical improvement and was discharged on oral warfarin and aspirin.
Figure 2: 2D echocardiography showing thrombus inside giant aneurysm in LAD

Click here to view

Case 3

A 7-year-old boy was referred for evaluation with a diagnosis of dilated cardiomyopathy. Chest X-ray showed huge cardiomegaly and 12 lead electrocardiogram surprisingly showed features of old anteroseptal myocardial infarction. Echocardiogram showed [Figure 3] akinesia of the left ventricular (LV) anterior wall and septum with a totally occlusive thrombus in the LMCA [Figure 3]. Focused interrogation revealed history suggestive of KD almost 1.5 years ago.
Figure 3: 2D echocardiography showing large organized thrombus occluding left coronary artery

Click here to view

Coronary angiogram confirmed multiple stenosis of LMCA with reduced perfusion. He underwent CABG with left internal mammary artery to LAD graft with satisfactory results. On follow–up, his ventricular function showed significant improvement. He is doing well on follow-up.

  Discussion Top

KD is a vasculitis that involves the medium-sized arteries of which coronary artery involvement is of particular concern.[1],[2] The incidence of coronary artery aneurysm (CAA) was reported to be 20%–26% in the pre-IVIG era which came down to 4%–5% since IVIG became the standard of care. Despite treatment with IVIG, 20% may develop transient coronary artery dilation, 5% may have aneurysms and 1% giant aneurysm.[3] Several predictors for coronary involvement have been studied. Age <1-year, nonresponse to one dose of IVIG, late administration of IVIG after 10 days of illness are risk factors for coronary complications[4],[5] Incomplete clinical manifestations are associated with delayed diagnosis and higher rate of coronary complications.[5] Laboratory markers such as, very high CRP, neutrophils, total bilirubin, transaminases, and pro brain natriuretic peptide and a fall in hemoglobin, albumin, sodium, and platelets are described as risk factors for IVIG resistance and risk factors for CAA.[6]

First two cases had many risk factors for the development of CAA namely, age of onset <6 months, incomplete clinical presentations, very high CRP, elevated liver enzymes. Due to incomplete clinical features and uncommon age group, KD was not initially considered. However, with the echocardiographic diagnosis of CAA, both of them were treated with IVIG. First case was responded to single dose of IVIG, but he had multiple CAA within the first 7 days of the illness which underscores the severity of the underlying inflammatory condition and emphasizes the need for early diagnosis in the below 6 months age. Our second case was resistant to first dose of IVIG but responded to the 2nd dose.

The treatment of acute coronary thrombosis in KD patients has not been tested in randomized controlled trials. Rather, recommendations for therapy are based on guidelines in adults with acute coronary syndrome (ACS) and small pediatric case series. The goal of therapy being re-establishing coronary artery patency and flow.[3] Compared with coronary artery thrombosis in an adult with atherosclerotic coronary artery disease, thrombus mass in KD patients is much greater. Intracoronary mechanical thrombosuction and intracoronary administration of thrombolytic agents are well described in adult literature. These interventions are limited by the size of the patient concern. Besides, as KD involves peripheral arterial system as well, obtaining femoral arterial access in an acute phase of illness in a newborn could be associated with higher risk of local complications. That's why we had chosen intravenous infusion of thrombolytic agent over intracoronary administration in our 2nd patient. However, intracoronary administration of thrombolytic medication in infant is described in literature.[7]

Scientific guideline published by the American Heart Association in 2017 recommends treatment for coronary artery thrombus in KD[3] tPA is recommended in such situations. For our 2nd patient, who was treated before 2017, we chose STK initially due to our familiarity with that drug. Later on, alteplase had to be used as there was no clinical improvement with STK.

Due to large thrombus burden in KD and tendency for rebound thrombosis, some clinicians preferred reduced-dose of thrombolytic therapy together with a glycoprotein IIb/IIIa inhibitor, such as abciximab. In adults with ACS, the inhibition of this receptor shown improved outcome, with and without the use of thrombolytic drugs. When echocardiographic surveillance in the 1st weeks of the illness reveals a small mural coronary artery thrombus that does not pose an urgent threat of occlusion, it may be reasonable to use only abciximab to prevent clot extension.[3],[8]

Percutaneous coronary intervention techniques used are those in the standard armamentarium of adult invasive cardiologist and include percutaneous transluminal coronary angioplasty (PTCA), rotational ablation, and stent placement. However, experience in pediatric age group is limited and coronary artery stenosis in patients with KD commonly involves severe calcifications, in contrast with adult coronary artery lesions, which primarily consist of atherosclerosis. Therefore, indication for catheter intervention in adult patients cannot be directly extrapolated to children with KD.[9],[10]

Surgical approach would be superior to interventional catheterization in patients with severe LV dysfunction and in cases with multiple, ostial, or long-segment stenoses.[10] Our 3rd patient presented with dilated and dysfunctional LV with large thrombus in LMCA. For which CABG was preferred over PTCA. A LIMA to LAD graft was used due to its growth potential with the somatic growth of the patient. Cardiac transplantation is reserved for patients with end stage ischemic cardiomyopathy, where coronary revascularization is not feasible.[3]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Arerugi 1967;16:178-222.  Back to cited text no. 1
Friedman KG, Gauvreau K, Hamaoka-Okamoto A, Tang A, Berry E, Tremoulet AH, et al. Coronary artery aneurysm in Kawasaki disease: Risk factors for progressive disease and adverse cardiac events in US population. J Am Heart Assoc 2016;5:e003289.  Back to cited text no. 2
McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment and long term management of Kawasaki disease. A scientific statement for health professionals from the American Heart Association. Circulation 2017;135:e927-99.  Back to cited text no. 3
Tang Y, Yan W, Sun L, Xu Q, Ding Y, Lv H. Coronary artery aneurysm regression after Kawasaki disease and associated risk factors: A 3-year follow-up study in East China. Clin Rheumatol 2018;37:1945-51.  Back to cited text no. 4
Song D, Yeo Y, Ha K, Jang G, Lee J, Lee K, et al. Risk factors for Kawasaki disease-associated coronary abnormalities differ depending on age. Eur J Pediatr 2009;168:1315-21.  Back to cited text no. 5
Song MS. Predictors and management of intravenous immunoglobulin-resistant Kawasaki disease. Korean J Pediatr 2019;62:119-23.  Back to cited text no. 6
Jone PN, Tapia D, Davidson J, Fagan TE, Browne L, Ing RJ, et al. Successful Treatment of Myocardial Infarction in an Infant with Kawasaki Disease. Seminars in Cardiothoracic and Vascular Anesthesia; 2015. p. 1-5.  Back to cited text no. 7
Lincoff AM, Kereiakes DJ, Mascelli MA, Deckelbaum LI, Barnathan ES, Patel KK, et al. Abciximab suppresses the rise in levels of circulating inflammatory markers after percutaneous coronary revascularization. Circulation 2001;104:163-7.  Back to cited text no. 8
Akagi T. Interventions in Kawasaki disease. Pediatr Cardiol 2005;26:206-12.  Back to cited text no. 9
Ishii M, Ueno T, Akagi T, Baba K, Harada K, Hamaoka K, et al. Guidelines for catheter intervention in coronary artery lesion in Kawasaki disease. Pediatr Int 2001;43:558-62.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
Case Reports
Article Figures

 Article Access Statistics
    PDF Downloaded54    
    Comments [Add]    

Recommend this journal