Approach
The main goal of treatment is to prevent cardiac complications, especially coronary artery aneurysms, by treating the inflammatory process as early as possible, in addition to treating the acute illness and reducing duration of inpatient stay.
During the acute phase, standard treatment includes intravenous immunoglobulin (IVIG), alongside aspirin.[1][13][28][32] Adjunctive anti-inflammatory therapy (e.g., corticosteroids, infliximab) should be considered early as part of initial therapy for high-risk patients and patients with Kawasaki shock syndrome.[13][28][32]
Risk factors for complications include age <6 months, Z score ≥2.5 on initial echocardiogram, and patients with severe inflammation (e.g., CRP >100 mg/L), persistent fever despite treatment, or persistently raised C-reactive protein (CRP).[28][50]
Presentation ≤10 days from onset or presentation >10 days from onset with evidence of ongoing inflammation
Standard treatment includes administering a single infusion of IVIG, early in the course of the disease within 10 days of the onset.[51] Treatment should be started as soon as a patient is diagnosed with either complete or incomplete KD.[1][13][28] IVIG is also indicated in patients who present after 10 days with KD, even if fever has resolved. This is considered to be the most current treatment regimen and has been successful in reducing the duration of fever and the prevalence of coronary artery aneurysms in KD.[32][51] Delaying treatment may lead to significant coronary artery aneurysms.[32] Live immunisations, such as measles, mumps varicella, should be deferred for 11 months after IVIG administration due to the risk of suppressing the immune response to the vaccine.[51] For patients at high risk of exposure to measles, the immunisation can be given and repeated at least 11 months after IVIG exposure.[13]
Aspirin should be used with IVIG therapy; it is thought to have an additive anti-inflammatory effect at higher doses, and can help prevent thrombosis via its anti-platelet effect at lower doses.[32] The optimal dose of aspirin for acute KD is unclear and this remains an active area of research.[28][32] In the US, the anti-inflammatory dose used in this setting has historically been higher than the dose used in Japan and Europe. However, increasing evidence suggests that higher doses may not improve outcomes.[28][32] The 2024 American Heart Association (AHA) guideline states that medium-dose aspirin during the acute phase is increasingly preferred in many centres.[28] The AHA recommends reducing the dose to antiplatelet dose levels 48-72 hours after fever resolves.[28] European guidelines also recommend medium-dose aspirin, but recommend reducing the dose 48 hours after fever resolves as long as clinical symptoms are improving and the CRP is falling.[13] Low-dose aspirin should be continued for 6-8 weeks after the acute episode and can then be stopped if the echocardiogram remains normal.[13][51] Patients with coronary artery aneurysms should be referred to cardiology. European guidelines note that patients with regressed aneurysms may have persistent endothelial function abnormalities and therefore ongoing low-dose aspirin should be considered depending on individual risk.[13]
Two-thirds of patients will be afebrile and improve within 24 hours of completion of the IVIG infusion, and 90% will be afebrile by 48 hours. This therapy regimen is effective in reducing the prevalence of coronary artery abnormalities from 20% to 25% down to 2% to 4%.[52]
Some patients may have persistent or recurrent fever 36-48 hours after a single dose of IVIG infusion. These patients are at increased risk of developing coronary artery abnormalities and may benefit from a second IVIG infusion, but guideline recommendations vary. The 2021 American College of Rheumatology/Vasculitis Foundation (ACR/VF) recommends that patients with acute KD and persistent fever after initial treatment with IVIG should receive a second course of IVIG rather than advancing therapy with corticosteroids.[32] However, the 2024 AHA guideline favours adding corticosteroids or a tumour necrosis factor (TNF)-alpha inhibitor (e.g., infliximab) for patients with IVIG resistance over giving a second dose of IVIG.[28] European guidelines recommend considering initiation of corticosteroids along with the second dose of IVIG in this situation.[13] A second dose of IVIG should be given with caution in patients with blood type A, B, or AB given the high risk of haemolysis.[28]
Monitoring response to treatment
The therapeutic target in KD is 'zero fever, zero CRP’. Resolution of fever should not be relied upon alone as a marker of treatment success because significant systemic inflammation may continue despite fever resolution. In the 48 hours following IVIG administration, patients should be afebrile and have a normal CRP (<10 mg/L) or CRP should be at least halving every 24 hours (as the half-life of CRP is 18 hours when hepatic production is ceased).[50]
A practical approach to monitoring response to treatment was published in 2022.[50] This approach was used by the Kawasaki Disease Coronary Artery Aneurysm Prevention trial, one multi-centre, randomised, open-label trial which investigated whether initial treatment with corticosteroids alongside IVIG and aspirin reduces incidence of coronary artery aneurysms compared with IVIG and aspirin alone.
Patients with high-risk features or refractory disease
Adjunctive anti-inflammatory therapy (e.g., a corticosteroid, infliximab) should be considered early as part of initial therapy alongside IVIG for high-risk patients and patients with Kawasaki shock syndrome.[13][28][32]
Anti-inflammatory therapy is also recommended as second-line treatment for patients with IVIG resistance or refractory disease.[13][28][32]
While the 2019 European guideline and 2021 ACR/VF guideline recommend corticosteroids as the preferred additional anti-inflammatory agent, the AHA recommends either corticosteroids or a TNF-alpha inhibitor as equal options.[13][28][32] Evidence is limited, but retrospective studies suggest that patients with a Z score ≥2.5 treated with IVIG plus either corticosteroids or infliximab have less progression in coronary artery aneurysm size and a higher likelihood of coronary artery aneurysm regression compared with those treated with IVIG alone.[28]
Various definitions for refractory KD have been postulated. The definition used in international guidelines and clinical trials is failure of resolution of fever within 36-48 hours of initial IVIG. However, it has been proposed that this definition should be broadened to include evidence of ongoing systemic inflammation within 48 hours of initial treatment to detect children with ongoing inflammation who are at risk of developing or worsening cardiac sequelae.[50]
IVIG resistance occurs in 10% to 20% of cases.[53] Patients with IVIG resistance are at a higher risk of developing coronary artery aneurysms.[13][54]
Outside Japan, clinical scores to predict IVIG resistance perform sub-optimally.[55][56][57] Therefore, clinicians elsewhere should assess the risk of IVIG resistance based on symptoms and laboratory values. Factors that increase the risk of IVIG resistance include: age <12 months; low serum sodium, haemoglobin, or albumin; high alanine transaminase, CRP, or bilirubin; and signs of shock or macrophage activation syndrome.[13][56]
A susceptibility gene on chromosome 19 which codes inositol 1,4,5-triphosphate 3-kinase C (ITPKC) has been shown to be significantly associated with IVIG resistance in KD patients and in those with coronary artery lesions.[11][54] It is probable that there are other genetic susceptibility genes which are yet to be identified.
Corticosteroids
While meta-analyses support the use of corticosteroids in patients with high-risk or refractory KD, individual studies have produced conflicting results.[58][59][60] Evidence comparing their efficacy with alternative anti-inflammatory therapies (e.g., TNF-alpha inhibitors) is limited, and guideline recommendations vary.
The 2024 AHA guideline recommends intensification of initial therapy with corticosteroids (or a TNF-alpha inhibitor) for patients with high-risk features, which they define as right coronary artery or left anterior descending Z score ≥2.5 at diagnosis, age <6 months, or patients in the high-risk category using the Son risk score (a risk score developed for prediction of coronary artery aneurysms in a North American population).[28] The AHA also recommends considering intensified initial therapy with IVIG plus a second anti-inflammatory agent for patients with Kawasaki shock syndrome. Second-line use of corticosteroids (or a TNF-alpha inhibitor) is recommended for patients with IVIG resistance.[28]
The 2021 ACR/VF guideline recommends that patients with acute KD who are at high risk of IVIG resistance or developing coronary artery aneurysms are given IVIG with adjunctive corticosteroids as initial therapy rather than IVIG monotherapy, with high-risk defined as a Z score greater than or equal to 2.5 for the left anterior descending coronary artery or right coronary artery at initial scan and aged <6 months, pending further studies to identify at risk patients in the US population.[32]
In Europe, corticosteroids are given as first-line to those with severe disease (high CRP >100 mg/L, persistently elevated CRP despite treatment, anaemia, hypoalbuminaemia, liver dysfunction, haemophagocytic lymphohistiocytosis or shock), those with evolving coronary or peripheral aneurysms at presentation with evidence of ongoing inflammation, and as rescue treatment in those with IVIG resistance (with either ongoing fever, persistent inflammation or ongoing clinical signs 48 hours after IVIG).[13][50] The 2019 European guidelines also recommend that corticosteroids are given to patients with proven IVIG resistance, Kobayashi score of 5 or more (if assessed with this tool), features of shock or macrophage activation syndrome, aged <1 year, or established presence of coronary or peripheral aneurysms with ongoing inflammation.[13]
Evidence suggests that corticosteroids are beneficial in refractory KD, and they appear to reduce the risk of heart problems after KD without causing any important adverse effects. One 2022 Cochrane review concluded that moderate-certainty evidence supports the use of corticosteroids in the acute phase of KD as it is associated with reduced coronary artery abnormalities, shorter duration of hospital stay, reduced inflammatory markers, and no serious adverse events or deaths when compared to those treated without corticosteroids.[59] However, 6 of the 8 trials were conducted in Japan or China, and none of the studies reported long-term (more than 1 year after diagnosis) outcomes.[59] Literature on the use of oral and intravenous pulse corticosteroid therapy in KD has produced conflicting results due to heterogeneity in the doses, regimens, and patient populations used in the studies.[61][62][63][64][65][66][67][68] Until more data are available, the subset of patients with KD that are IVIG-resistant and/or with life-threatening complications should be treated with corticosteroid therapy.[59][69] [
]
Further prospective, multicentre, randomised controlled trials are needed to determine the efficacy of pulsed intravenous or oral doses of corticosteroids in the treatment of IVIG-resistant KD, and also for unselected cases of KD.
TNF-alpha inhibitors
Some centres use a TNF-alpha inhibitor (e.g., infliximab, etanercept) as part of intensified initial therapy for patients with high-risk features, and the 2024 AHA guideline supports this approach.[28] TNF-alpha inhibitors may also be used in patients refractory to IVIG and corticosteroids.[28][70]
The 2021 ACR/VF guideline recommends that patients with acute KD who are at high risk of IVIG resistance or developing coronary artery aneurysms are given IVIG with adjunctive immunomodulatory/immunosuppressive agents (e.g., infliximab, anakinra, or ciclosporin) as initial therapy rather than IVIG monotherapy, if corticosteroids are contraindicated.[32]
The 2019 European SHARE guidelines also recommend consideration of using TNF-alpha inhibitors in patients with persistent inflammation despite IVIG, aspirin, and corticosteroids.[13]
One 2021 multi-centre trial compared a second IVIG dose with infliximab in IVIG-resistant children. Infliximab treatment resulted in shorter duration of fever, reduced need for additional therapy, less severe anaemia, and shorter hospitalisation compared with a second IVIG dose.[71] There was no difference between inflammatory markers or rates of coronary artery abnormalities between the groups, but the study was underpowered to assess effect on coronary arteries because it excluded patients who received primary intensification of treatment for coronary artery abnormalities.[71]
One Cochrane review looked at TNF-alpha inhibitors used after IVIG or as initial treatment in five trials including 494 children. Four of the trials used infliximab and one used etanercept. The authors found low-certainty evidence that TNF-alpha inhibitors reduced treatment resistance and were associated with fewer infusion reactions compared with no treatment or additional IVIG. No differences were found in the incidence of coronary artery abnormalities or infections between groups.[72]
In one meta-analysis of eight studies of infliximab therapy used after IVIG or as initial treatment, infliximab significantly reduced treatment resistance when used as initial treatment compared to IVIG alone, and improved clinical course when used in IVIG resistant patients compared to repeat IVIG.[73]
One network meta-analysis comparing different combinations of pharmacological interventions for KD found that the combination of medium dose IVIG, aspirin, and infliximab resulted in the shortest duration of fever and lowest incidence of coronary artery abnormalities in the initial stage of KD. In the refractory phase, IVIG and pulsed-dose corticosteroids and high-dose IVIG and ciclosporin were the combinations associated with the most rapid resolution of fever and lowest incidence of coronary artery abnormalities.[58] Heterogeneity of underlying studies may have introduced bias in these results.
Two meta-analyses compared IVIG, methylprednisolone, and infliximab in patients with refractory KD. They included many of the same studies but came to different conclusions. One study reported that infliximab and methylprednisolone were significantly more effective in resolving fever than a second dose of IVIG.[74] The other study reported that infliximab was significantly more effective in resolving fever than both methylprednisolone and a second dose of IVIG.[75] Neither study found any differences in the rates of coronary artery lesions between treatment groups.
One systematic review and meta-analysis found that although monoclonal antibodies and anti-cytokine biologicals were not effective in reducing the frequency of coronary artery abnormalities in KD patients, the frequency of IVIG resistance may be reduced compared with conventional monotherapy with IVIG.[76]
Other immunomodulatory drugs or plasma exchange
Patients with refractory KD in whom a second dose of IVIG, corticosteroids, and infliximab have failed may receive an alternative immunomodulatory drug. There is no consensus or evidence as to whether ciclosporin, anakinra, or cyclophosphamide should be used after other treatments fail and cases should be discussed with a specialist centre.
One network meta-analysis found that the combination therapy with high-dose IVIG and corticosteroids or ciclosporin may have an additional effect on improving the rate of decline of fever and lowering the incidence rate of coronary artery abnormalities in children with refractory KD. However, the authors caution that new randomised trials would be required to support the results.[58]
There has been abundant evidence from human patients, genetic studies, and experimental mouse models that support the involvement of interleukin-1b (IL-1b) in the pathogenesis of KD. Anakinra is a competitive inhibitor to both IL-1a and IL-1b and acts by blocking the IL-1 binding to the IL-1 receptor. The Kawakinra study, a phase 2 clinical trial, aimed to determine the efficacy, safety and tolerability of blocking IL-1 signalling in patients with acute KD who are unresponsive to treatment with IVIG. While this study supported the early use of anakinra in cases refractory to IVIG, the effect on the KD symptoms and inflammation parameters took an average of 14 days to achieve. Ultimately, the study lays down the groundwork for controlled clinical trials to fully further evaluate the efficacy of anakinra as a first line treatment for KD.[77]
Very rarely, plasma exchange may be considered for patients with refractory KD when all other treatment options are ineffective.[1][13] Evidence to support the use of plasma exchange for KD is limited, and this option should only be considered on a case-by-case basis in consultation with a KD specialist.[13] One retrospective study from Japan assessed the benefit of adding plasma exchange rescue (PER) to KD patients who were IVIG and infliximab non-responders. The study showed that the addition of PER improved fever, other acute symptoms, laboratory data, and coronary outcomes. These results remain uncertain pending future randomised trials.[78]
Presentation >10 days from onset without evidence of ongoing inflammation
Patients with KD that present without persistent fever after day 10, and when their acute phase markers (ESR and/or CRP) are normal, are regarded as without risk of developing coronary aneurysms.
If their initial and subsequent echocardiograms are normal, they should be treated with low-dose aspirin until 6-8 weeks from the onset. If echocardiogram at 8 weeks is normal, low-dose aspirin can be discontinued.
However, if diagnosed after day 10 with evidence of elevated ESR or CRP and/or coronary abnormalities on echocardiogram, patients should be treated as detailed above for patients with evidence of ongoing inflammation.
Long-term management
All patients with current or previous aneurysms following KD require lifelong follow-up by a cardiology team within a specialist KD service. Their risk of future cardiac events is proportional to the degree of cardiac pathology remaining after the acute illness has resolved.[12] European recommendations suggest that ECG and echocardiography should be performed every 3-6 months in children with coronary artery aneurysms, depending on their severity.[13]
The 2017 AHA guidelines included a stratification system based on maximum Z-score (ever measured) and current Z-score (during long-term follow up) to categorise patients by their risk level for development of myocardial ischaemia; this was updated by the AHA in 2024 to reflect new data.[1][28]
Many of the concepts in the recommendations for each risk stratum are similar, but differ in timing. For brevity, any specific differences are noted under each risk stratum separately below, but the general concepts are:
Promote healthy lifestyle and activity at every visit.
If a patient cannot tolerate or is resistant to aspirin, use an alternative antiplatelet agent such as clopidogrel.[28] For dual antiplatelet therapy, if the patient is already on clopidogrel, consider adding dipyridamole as an alternative. Dipyridamole is generally no longer used for long-term thromboprophylaxis, but it is an alternative to aspirin in patients who are taking ibuprofen, resistant or allergic to aspirin, or at risk of Reye syndrome.[28]
Cardiovascular risk factor assessment should include blood pressure, fasting lipid profile, body mass index, waist circumference, diet, activity, and smoking.
Restrict high-contact activities in patients on anticoagulation or dual antiplatelet therapy. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias.
Follow-up cardiology assessment should include history and physical examination, echocardiography, and electrocardiography for all patients.
Patients with coronary artery aneurysms require periodic surveillance for inducible myocardial ischaemia with stress echocardiography, stress with magnetic resonance imaging, stress nuclear medicine, or positron emission tomography. Patients who had coronary artery aneurysms (small, medium, large, or giant) that have regressed still require ischaemic testing. Imaging modality depends on the patient’s age and institutional expertise. Stress echocardiography may be preferred for patients aged >7 years; if available, magnetic resonance stress imaging may be useful for children who are too young to exercise.[28]
Further imaging includes angiography by computed tomography, magnetic resonance imaging, or invasive angiography.
Computed tomography angiography (CTA) is typically preferred for detection and follow-up of coronary artery aneurysms, but the choice of imaging modality depends on the clinical context and local availability.[28][37] CTA can be performed in children with relatively low radiation exposure if obtained at an institution with proper expertise.
Reproductive counselling is important for patients with a history of KD, but evidence to guide recommendations is limited.[1][79] Based on clinical experience in practice, patients with significant cardiac sequelae who are taking systemic anticoagulants or dual antiplatelet therapy should avoid oestrogen-containing oral contraceptives when possible due to the increased risk of thrombosis. In patients with residual coronary abnormalities, pregnancy should be supervised by a multidisciplinary team including a cardiologist who is familiar with management of patients with KD. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.[1]
Beta-blockers are not part of the standard treatment of KD. They should be considered on an individual basis if there is concern for myocardial ischaemia, particularly for patients with persistent large or giant aneurysms and/or any region of stenosis prior to aneurysm. However, data are limited, and additional features beyond aneurysm size may indicate an increased risk of myocardial ischaemia.[1] The 2024 AHA guideline suggests that beta-blockers may be considered for patients with medium, large or giant aneurysms.[28] Patients with aneurysms that regress to diffuse mild dilation with no discrete aneurysm likely have a lower risk of ischaemia.
The use of statin therapy is becoming more common. See Emerging.
No involvement (Z score always <2)
Give low-dose aspirin after the acute episode, but discontinue aspirin after 6 weeks. Consider follow-up at 4-6 weeks after the acute episode if coronary artery imaging is suboptimal or laboratory markers of inflammation are abnormal at 1-2 weeks. Discharge patients from cardiology follow-up between 4 weeks and 12 months after disease onset, depending on individual clinical context.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease.
Counsel patients about contraception and pregnancy as you would patients without KD.
Dilation only (Z scores ≥2.0 but <2.5)
Give low-dose aspirin, but discontinue it 6 weeks after the episode of acute disease if Z score and laboratory tests are normal. Consider follow-up at 6 weeks after the acute disease episode if there are abnormalities at 1-2 weeks. Discharge from cardiology if luminal dimensions have returned to normal by 12 months after disease onset. If dilation persists, continue follow-up every 2-5 years.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease.
Advise patients about contraception and pregnancy as you would advise patients without KD.
Small aneurysms (Z score ≥2.5 to <5.0)
Current or persistent small aneurysms
Treat these patients with low-dose aspirin. Follow up at 6 weeks after the acute disease episode, assess after 6 months and 1 year, and follow up every year thereafter.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 3-5 years, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, with further imaging with angiography every 3-5 years.
Advise patients about contraception and pregnancy as you would patients without KD.
Regression to normal Z score or dilation only
Consider treatment with low-dose aspirin, although it is reasonable to discontinue aspirin after coronary arteries regress to normal. Assess these patients at 6 weeks and 1 year after the acute episode. Patients may be discharged after 5 years if stress test and coronary CTA are normal.
Assess cardiovascular risks every 2 years. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 5 years, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, and consider further imaging with angiography only if there is evidence for inducible myocardial ischaemia or ventricular dysfunction.
Advise patients about contraception and pregnancy as you would patients without KD.
Medium aneurysms (Z score ≥5 to <10, with an absolute luminal dimension <8 mm)
Current or persistent medium aneurysms
Treat with low-dose aspirin. Consider dual antiplatelet therapy with an additional antiplatelet agent. Treatment with a beta-blocker may be considered if there is concern for myocardial ischaemia.
Patients should be reviewed at 6 weeks after the acute disease episode, then assessed after 3 months, 6 months, and 1 year, and followed up every 12 months thereafter.
Assess cardiovascular risks at least once and ideally at least 1 year from the episode of acute disease. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 2-5 years, or sooner if the patient has symptoms suggestive of ischaemia or signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, with further imaging with angiography every 2-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias. Restrict activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet therapy.
Based on clinical experience in practice, patients with significant cardiac sequelae who are taking dual antiplatelet therapy should avoid oestrogen-containing oral contraceptives when possible due to the increased risk of thrombosis. In patients with residual coronary abnormalities, pregnancy should be supervised by a multidisciplinary team including a cardiologist who is familiar with management of patients with KD. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.[1]
Regression to small aneurysms
Treat with low-dose aspirin. Treatment with a beta-blocker may be considered if there is concern for myocardial ischaemia. Follow up patients at 6 weeks, 6 months, 12 months, and yearly thereafter.
Assess cardiovascular risks every year. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 3-5 years, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, with further imaging with angiography every 3-5 years.
Advise patients as per normal about contraception, but recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.
Regression to normal Z score or dilation only
Treat with low-dose aspirin. Do not use an additional antiplatelet agent unless there is evidence of inducible myocardial ischaemia. Follow up at 6 weeks, 6 months, 12 months, and every 2 years thereafter.
Assess cardiovascular risk every 2 years. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 4-5 years, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline. Further imaging with angiography may not be needed in the absence of evidence of inducible myocardial ischaemia.
No restrictions regarding contraception and pregnancy are applicable to this group of patients.
Large and giant aneurysms (Z score ≥10 or absolute dimension ≥8 mm)
Current or persistent large and giant aneurysms
Treat with low-dose aspirin. Use warfarin to achieve a target international normalised ratio of 2-3, or a low molecular weight heparin (LMWH) to achieve anti-factor Xa levels of 0.5 to 1.0 units/mL. Consider dual antiplatelet therapy with aspirin and an additional antiplatelet agent together with warfarin, LMWH, or a direct oral anticoagulant (DOAC) such as apixaban for thromboprophylaxis in the setting of very extensive or distal coronary artery aneurysms, or if there is a history of coronary artery thrombosis. Consider treatment with a beta-blocker. Assess patients at 6 weeks, and then at 3, 6, 9, and 12 months after the episode of acute disease in the first year, and every 6-12 months thereafter.
Assess cardiovascular risks every 6-12 months. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 6-12 months, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider further imaging with coronary CTA for diagnostic and prognostic purposes within 2-6 months and every 1-5 years thereafter. Invasive coronary angiography may be considered.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Based on clinical experience in practice, patients with significant cardiac sequelae who are taking systemic anticoagulants or dual antiplatelet therapy should avoid oestrogen-containing oral contraceptives when possible due to the increased risk of thrombosis. In patients with residual coronary abnormalities, pregnancy should be supervised by a multidisciplinary team including a cardiologist who is familiar with management of patients with KD. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.[1]
Regression to medium aneurysms
Treat with low-dose aspirin. Dual antiplatelet therapy with an additional agent may be considered. Anticoagulation with warfarin, LMWH, or a DOAC may be considered for patients who are at risk for thrombosis; specialist consultation may be helpful when the trade-offs between thrombosis and bleeding risk are difficult to balance. Reduction in the lumen size can occur due to thrombosis, in which case anticoagulation is indicated. Consider treatment with a beta-blocker. Assess the patient every 6-12 months.
Assess cardiovascular risks every 12 months. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 2-5 years, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, with further imaging with angiography every 2-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Based on clinical experience in practice, patients with significant cardiac sequelae who are taking systemic anticoagulants or dual antiplatelet therapy should avoid oestrogen-containing oral contraceptives when possible due to the increased risk of thrombosis. In patients with residual coronary abnormalities, pregnancy should be supervised by a multidisciplinary team including a cardiologist who is familiar with management of patients with KD. It may be necessary to alter thromboprophylaxis during pregnancy and delivery.[1]
Regression to small aneurysms
Treat with low-dose aspirin; dual therapy with an additional antiplatelet agent may be considered. Consider treatment with a beta-blocker. Assess the patient at 6 weeks, 3 months, 6 months, 9 months, 12 months, and then yearly.
Assess cardiovascular risks every 12 months. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 3-5 years, or sooner if the patient has symptoms suggestive of ischaemia or has signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, with further imaging with angiography every 3-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Advise patients as per normal about contraception, but recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist, and that thromboprophylaxis may be altered during pregnancy and delivery.
Regression to normal Z score or dilation only
Treat with low-dose aspirin. Dual antiplatelet therapy and thromboprophylaxis are generally not indicated for patients with large or giant aneurysms that have regressed to normal or dilation only.[28] However, patient and coronary artery characteristics may influence decisions about thromboprophylaxis.[1] Follow up at 6 weeks, 3 months, 6 months, 9 months, 12 months, and then every 1-2 years.
Assess cardiovascular risks every 2 years. Obtain a follow-up fasting lipid profile.
Assess for inducible myocardial ischaemia every 3-5 years, if the patient has symptoms suggestive of ischaemia or signs suggestive of ventricular dysfunction. Consider coronary CTA at 1 year as baseline, with further imaging with angiography every 3-5 years.
Advise that participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischaemia or exercise-induced arrhythmias. Restrict or modify activities involving a risk of bodily contact, trauma, or injury for patients taking dual antiplatelet or anticoagulation therapy.
Advise patients about contraception as per normal, but recommend that pregnancy is supervised by a multidisciplinary team including a cardiologist, and that thromboprophylaxis may be altered during pregnancy and delivery.
Similarly the SHARE guidelines recommend a repeat echocardiogram:[13]
In patients in whom the initial echocardiogram was normal and the systemic inflammatory process has improved at 2 weeks after initial IVIG
In all patients with KD at 6-8 weeks
In those with ongoing active inflammation (symptoms or signs of KD and/or raised inflammatory markers) at least weekly to monitor for cardiac complications
In those with abnormalities on initial echocardiogram at least weekly to ensure stabilisation
In those with coronary artery aneurysm 3-6 monthly.
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