Kawasaki disease and giant coronary artery aneurysms: the role of echocardiography from diagnosis through follow-up

Background: Long-term outcomes and life expectancy for children with a previous history of Kawasaki disease (KD), particularly those with coronary artery aneurysms (CAA), remain to be determined. Methods: An inception cohort of all patients with KD assessed at The Hospital for Sick Children in Toronto between 1978 and 2013 was assembled. Patient outcomes were obtained throughout their pediatric and adult clinical follow-up as long as available. Prevalence of outcomes over time was modelled with Kaplan-Meier survival curves. Life tables from Statistics Canada were used to obtain age/gender specific cumulative mortality for the general population. Results: The cohort included 2,623 KD patients, of whom 410 (16%) had coronary artery involvement (215 dilatation, 57 non-giant CAA and 138 giant CAA). Average follow-up for patients with coronary artery involvement was 6.7 years (13.3 years for giant CAA); 57 and 34 patients had at least 15 and 25 years of follow-up, respectively. No patients with coronary artery dilatation or non-giant CAA had revascularization or a myocardial infarct. Freedom from revascularization (14 events) for patients with giant CAA was 90±6%, 87±7% and 80±13% at 10, 20 and 40 years of follow-up. Freedom from myocardial infarct (11 events) was 94±4%, 92±5% and 89±7% at 5, 20 and 40 years. For patients without coronary artery involvement, 3 (0.1%) deaths were recorded, one secondary to complications of macrophage activation syndrome during the acute phase of KD and 2 from cancer. No deaths were noted for patients with coronary artery dilatation or non-giant CAA, although clinical follow-up was more limited. For patients with giant CAA, 3 deaths (2.2%) were noted, 2 related to CAA complications and 1 from non-medical cause. Cumulative mortality for patients with giant CAA was 1.5% at 10 years of age (expected mortality 0.7%, HR: 2.2 (0.3-11.5), p=0.08) and 3.1% at 40 years of age (expected 2.3%, HR: 1.3 (0.4-4.0), p=0.37). Conclusions: Despite risks of myocardial infarction and revascularization, patients with giant CAA had life-expectancy similar to that of the general population up to the fourth decade of life. Additional follow-up will be necessary to determine if these trends continue into later decades.

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Multidetector Row Computed Tomography for Follow-Up of Patients with Coronary Artery Aneurysms Due to Kawasaki Disease

Pediatric Cardiology ◽

10.1007/s00246-003-0559-3 ◽

2004 ◽

Vol 25 (1) ◽

pp. 35-39 ◽

Cited By ~ 29

Author(s):

S. Sohn ◽

H. S. Kim ◽

S. W. Lee

Keyword(s):

Computed Tomography ◽

Coronary Artery ◽

Kawasaki Disease ◽

Multidetector Row Computed Tomography ◽

Coronary Artery Aneurysms ◽

Multidetector Row

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Coronary aneurysms, myocardial dysfunction, and shock in a COVID-19 child, role of ECMO, immunomodulation, and cardiac CT

Cardiology in the Young ◽

10.1017/s1047951121000147 ◽

2021 ◽

pp. 1-5

Author(s):

Muhammad R. Mustafa ◽

Michael J. Carter ◽

James Wong ◽

Aaron Bell ◽

Caner Salih

Keyword(s):

Case Report ◽

Coronary Artery ◽

Cardiac Ct ◽

Myocardial Dysfunction ◽

Rare Complication ◽

Coronary Artery Aneurysms ◽

Coronary Aneurysms ◽

Inflammatory Syndrome

Abstract Coronary artery aneurysms in children were observed as a rare complication associated with coronavirus disease 2019 (COVID-19). This case report describes the severe end of the spectrum of the new multisystem inflammatory syndrome in a 12-year-old child with coronary aneurysms, myocardial dysfunction, and shock, managed successfully with extracorporeal membrane oxygenation support and immunomodulation therapy. This report also highlights the additional benefits of cardiac CT in the diagnosis and follow-up of coronary aneurysms.

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Abstract O.71: Thrombosis and Thromboprophylaxis for Patients with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Study from the North American Kawasaki Disease Registry

Circulation ◽

10.1161/circ.131.suppl_2.o71 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Cedric Manlhiot ◽

William T Mahle ◽

Kevin D Hill ◽

Jennifer S Li ◽

Dawn Tucker ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

North American ◽

Event Rate ◽

Disease Registry ◽

Coronary Artery Aneurysms ◽

The North ◽

Thrombotic Complications

Background: Children with giant coronary artery aneurysms (CAA) after Kawasaki disease (KD) are at substantial risk of thrombosis. There are currently no evidence-based guidelines for optimal thromboprophylactic therapy in these children. Methods: The North American Kawasaki Disease Registry was queried to identify all patients with giant CAA (maximum coronary artery z-score >10) and their antithrombotic therapy. Freedom from thrombosis was modelled using the Kaplan-Meier method; thrombotic complication rate was calculated per patient-year/month of follow-up. Results: n=202 patients with giant CAA were included, of whom 28 (14%) experienced either coronary artery thrombosis with or without myocardial infarction. Freedom from thrombotic complications was 92%, 85% and 79% at 3 months, 5 and 10 years after diagnosis, respectively. Non-pharmacological factors associated with increased risk of thrombotic complications included higher maximum coronary artery z-scores (HR: 1.7/+10 SD, p<0.001), higher number of coronary artery branches with giant CAA (HR: 2.6/branch, p<0.001), higher number of discrete CAA (HR: 1.4/aneurysm, p=0.001) and presence of complex CAA (involving the bifurcation or non-discrete; HR: 3.0, p=0.05). A total of 982 patient-years of follow-up were available for analysis (11% low molecular weight heparin (LMWH), 32% warfarin, 57% antiplatelet alone). All patients were maintained on ASA, with 47 patients (23%) also receiving clopidogrel. Patients while on LMWH had the highest event rate, at 1 event per 13 patient-years, compared to 1 per 39 on warfarin and 1 per 33 on no anticoagulant. However, LMWH was predominantly prescribed immediately after the acute phase, which is also the highest risk phase for thrombosis. When limiting analysis to events within 3 months of the acute phase, patients on LMWH had the lowest event rate at 1 per 46 patient-months, compared to 1 per 27 on warfarin and 1 per 33 on no anticoagulant (p=NS). Conclusions: Current thromboprophylaxis strategies in patients with giant CAA have suboptimal efficacy, and residual thrombosis risk persists. New anticoagulants/antiplatelet agents should be assessed in this population to determine if they provide better, safer and more tolerable thromboprophylaxis.

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Faculty Opinions recommendation of ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1112163.568149 ◽

2008 ◽

Author(s):

Dan Lipsker

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Disease Susceptibility ◽

Functional Polymorphism ◽

Coronary Artery Aneurysms

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Coronary artery aneurysms: outcomes following medical, percutaneous interventional and surgical management

Open Heart ◽

10.1136/openhrt-2020-001440 ◽

2021 ◽

Vol 8 (1) ◽

pp. e001440

Author(s):

Shameer Khubber ◽

Rajdeep Chana ◽

Chandramohan Meenakshisundaram ◽

Kamal Dhaliwal ◽

Mohomed Gad ◽

...

Keyword(s):

Coronary Artery ◽

Surgical Management ◽

Medical Management ◽

Cox Regression ◽

Artery Bypass ◽

Management Strategies ◽

Coronary Artery Aneurysms ◽

Kaplan Meier

BackgroundCoronary artery aneurysms (CAAs) are increasingly diagnosed on coronary angiography; however, controversies persist regarding their optimal management. In the present study, we analysed the long-term outcomes of patients with CAAs following three different management strategies.MethodsWe performed a retrospective review of patient records with documented CAA diagnosis between 2000 and 2005. Patients were divided into three groups: medical management versus percutaneous coronary intervention (PCI) versus coronary artery bypass grafting (CABG). We analysed the rate of major cardiovascular and cerebrovascular events (MACCEs) over a period of 10 years.ResultsWe identified 458 patients with CAAs (mean age 78±10.5 years, 74.5% men) who received medical therapy (N=230) or underwent PCI (N=52) or CABG (N=176). The incidence of CAAs was 0.7% of the total catheterisation reports. The left anterior descending was the most common coronary artery involved (38%). The median follow-up time was 62 months. The total number of MACCE during follow-up was 155 (33.8%); 91 (39.6%) in the medical management group vs 46 (26.1%) in the CABG group vs 18 (34.6%) in the PCI group (p=0.02). Kaplan-Meier survival analysis showed that CABG was associated with better MACCE-free survival (p log-rank=0.03) than medical management. These results were confirmed on univariate Cox regression, but not multivariate regression (OR 0.773 (0.526 to 1.136); p=0.19). Both Kaplan-Meier survival and regression analyses showed that dual antiplatelet therapy (DAPT) and anticoagulation were not associated with significant improvement in MACCE rates.ConclusionOur analysis showed similar long-term MACCE risks in patients with CAA undergoing medical, percutaneous and surgical management. Further, DAPT and anticoagulation were not associated with significant benefits in terms of MACCE rates. These results should be interpreted with caution considering the small size and potential for selection bias and should be confirmed in large, randomised trials.

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