Abstract O.58: Medium-term Outcomes of Coronary Artery Aneurysms after Kawasaki Disease: A Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Brian W McCrindle ◽  
Cedric Manlhiot ◽  
Kristen Sexson ◽  
Pei-Ni Jone ◽  
Mathew Mathew ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
North American ◽  
Z Score ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
Luminal Diameter ◽  
The North ◽  
Increased Risk ◽  
Over Time

Background: One of the main impediments to conceiving and planning studies in children with coronary artery aneurysms (CAA) after Kawasaki disease (KD) is the lack of normative data regarding the prevalence of outcomes over time and risk factors. Methods: The North American Kawasaki Disease Registry was used to determine the prevalence of multiple clinically important outcomes of CAA after KD. All analyses were stratified by severity of CAA (small CAA with z-score = 2.5-5, medium with z-score = 5-10 and giant with z-score >10). All analyses were performed using non-parametric survival analysis. Results: n=621 patients submitted to the Registry had complete follow-up data and were included in the analysis (280 [45%] small CAA, 139 [22%] medium and 202 [33%] giant). Time-related freedom from multiple outcomes stratified by type of CAA are reported in the Table. Reduction in z-scores was strongly associated with the initial size of the lesion, with smaller lesions being more likely to decrease to a normal dimension over time. Thrombosis and stenosis were infrequent in patients without giant CAA. For those patients with giant CAA, the risk of thrombosis, myocardial infarction, angiographically-confirmed stenosis and revascularization was substantial and persisted up to 10 years after diagnosis. In addition to larger luminal diameter, other factors associated with increased risk of adverse outcomes included larger CAA longitudinal area and complex CAA (vs. isolated lesions). Conclusions: Only patients with giant CAA are at substantial risk of adverse clinical outcomes; future trials of pharmacological therapy targeting thrombosis and stenosis risk should focus on these patients.

Abstract 221: Use of Statins in Patients with Coronary Artery Aneurysms: A Pilot Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Michael Khoury ◽  
Michael A Portman ◽  
Cedric Manlhiot ◽  
Anne Fournier ◽  
Rejane F Dillenburg ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Pilot Study ◽  
Kawasaki Disease ◽  
Linear Regression ◽  
North American ◽  
Z Score ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
The North ◽  
Significant Difference

Background: Statins have been considered as therapy for children with coronary artery aneurysms (CAA) after Kawasaki disease (KD), due to potential beneficial pleiotropic effects which might influence chronic vascular processes and inflammation. Methods: The North American Kawasaki Disease Registry was queried to identify patients who have received statins in the first 6 months following the convalescent phase of KD. Each identified patient was matched by age, gender and CAA z score to 3 patients who were statin-naïve (controls). Linear regression models adjusted for repeated measures and maximum coronary involvement were used to determine an association of statin use with longitudinal changes in coronary artery diameter z-score. Kaplan-Meier analysis was used to compare freedom from angiographically-confirmed stenosis or interventions. Results: Of 29 patients with KD and CAA (maximum coronary artery z-score >10) who received statins at any time (of n=621, 5%), 10 (9 males) patients were started within 6 months of the acute KD episode. The mean age at KD was 6.3±3.4 years (5.4±3.5 for controls, p=0.57). Mean maximum CAA z-score was 36±14 (vs. 29±16, p=0.20); 90% of statin patients and 87% of matched controls had CAAs in 3 or more branches. Linear regression analysis of 442 serial echocardiograms showed that maximum CAA z-score decreased by -1.5 (95%CI: -2.7; -0.4) SD/year (p=0.008) for control patients compared to -2.9 (95%CI: -4.4; -1.4) SD/year (p<0.001) for statin treated patients. The difference between the rate of change of CAA z-score for statin vs. control patients did not reach statistical significance (controls vs. statins: +1.4 SD/year, 95%CI: -0.6; +3.4, p=0.18). n=7 patients (3 on statin, 4 controls) developed stenosis or had revascularization, with no significant difference between groups (HR for statin group: 2.2 (0.4-11.4), p=0.41). Conclusions: This underpowered pilot study suggests that equipoise likely exists with regards to statin therapy in children with KD and CAA, and that a formal registry-nested trial might be considered.

Abstract 225: Variations in Pharmacological Management of Children with Coronary Artery Aneurysms after Kawasaki Disease: A Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Elif Seda Selamet Tierney ◽  
Andrew S Mackie ◽  
Brian W McCrindle ◽  
Mathew Mathew ◽  
Kathryn R Armstrong ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
North American ◽  
Beta Blockers ◽  
Practice Variation ◽  
Z Score ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
Pharmacological Management ◽  
The North

Background: The pharmacological management of coronary artery aneurysms (CAA) associated with Kawasaki disease (KD) is based on imperfect evidence, which may lead to considerable practice variation. Methods: Pharmacological management of patients included in the North American Kawasaki Disease Registry was reviewed. The Registry included data for 621 patients with CAA after KD (280 patients with maximum CAA z-score between 2.5-5.0, 139 with z-score 5.0-10.0 and 202 with z-score >10.0) followed at 20 medical centers. Practice variation regarding acute treatment, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy and anticoagulation were assessed. Results: Considerable practice variation existed between centers. During the acute phase, 93% of patients received at least one dose of IVIG (range: 80-100%), with 23% (range: 12-50%) receiving additional immunomodulatory treatment (22% additional IVIG, 17% steroids, 4% infliximab). Use of a 3 rd course of IVIG was infrequent (2%). All centers reported using additional IVIG or steroids for IVIG-resistant patients, but only 6 centres reported any experience with infliximab (2 commonly, 4 infrequent). Routine use of non-steroidal anti-inflammatory agents was limited to 2 centres, with 4 additional sites reporting infrequent use (10% of patients). Statins (5%), beta blockers (4%) and abciximab (3%) were mostly used by a single centre and was limited to patients with giant CAAs. Aspirin was the primary antiplatelet modality for 97% of patients, clopidogrel (10% of all patients, 23% in giant CAA) was routinely prescribed to patients with giant CAAs at 6 centres, with 2 more centres reporting infrequent use and the remainder not reporting any use. For patients with giant CAA (z-score>10.0), 46% were maintained on an antiplatelet agent alone, 17% additionally were on low molecular weight heparin(LMWH), 12% on warfarin and 25% had initially received LMWH and were later switched to warfarin. Conclusions: Given the important variations in management between centres and the poor evidence base, randomized controlled trials examining outcomes and nested in a high-quality collaborative registry may be an efficient strategy to address this gap.

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 ◽  
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.

scholarly journals Recurrent Kawasaki Disease: A Case Report of Three Separate Episodes at >4-Year Intervals

Children ◽  
2018 ◽  
Vol 5 (11) ◽  
pp. 155 ◽  
Author(s):  
Nikita Goswami ◽  
Katherine Marzan ◽  
Elizabeth De Oliveira ◽  
Sharon Wagner-Lees ◽  
Jacqueline Szmuszkovicz
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
High Dose ◽  
Z Score ◽  
Coronary Aneurysms ◽  
Increased Risk ◽  
The Right ◽  
Work Up ◽  
High Dose Aspirin

Kawasaki disease (KD) is a self-limited systemic vasculitis, most often occurring in children 1–5 years old. It has a 2% recurrence rate and is associated with coronary aneurysms (CA), which can develop within two weeks of onset. A 25% increased risk is noted in patients who are recalcitrant to treatment. We describe a patient with recurrence of KD three times, approximately four years apart. A 10-year-old female with two previous episodes of KD, at 11 months and five years of age), in which she met five out of five criteria for KD and had no coronary involvement, presented with 15 days of fever, conjunctivitis and mucocutaneous changes. Infectious work-up was negative, and she was diagnosed with incomplete KD meeting three out of five criteria. An echocardiogram (ECHO) on day 12 revealed dilation of the right coronary artery (RCA) and left coronary artery (LCA). Treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin was started at an outside hospital. After transfer, serial ECHOs showed evolving coronary aneurysms, left anterior descending (LAD) z-score + 8.2 and RCA z-score + 4.0. She received 10 mg/kg infliximab (day 18) and began clopidogrel. A cardiac MRI (day 20) demonstrated progression of the LAD aneurysm, with a z-score + 13, and warfarin was started. To our knowledge, this is the first report of recurrent KD occurring three times at ~5 year intervals.

scholarly journals Medium‐Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry

2020 ◽  
Vol 9 (15) ◽  
Author(s):  
Brian W. McCrindle ◽  
Cedric Manlhiot ◽  
Jane W. Newburger ◽  
Ashraf S. Harahsheh ◽  
Therese M. Giglia ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Medium Term ◽  
Disease Registry ◽  
Coronary Artery Aneurysms

scholarly journals CT Angiography or Cardiac MRI for Detection of Coronary Artery Aneurysms in Kawasaki Disease

2021 ◽  
Vol 9 ◽  
Author(s):  
Diana van Stijn-Bringas Dimitriades ◽  
Nils Planken ◽  
Irene Kuipers ◽  
Taco Kuijpers
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Cardiac Mri ◽  
Diagnostic Yield ◽  
Imaging Modality ◽  
Computed Tomographic Angiography ◽  
Single Center ◽  
Coronary Artery Aneurysms ◽  
Luminal Diameter ◽  
Diagnostic Potential

Background: Kawasaki disease (KD) is an acute vasculitis that mainly affects the coronary arteries. This inflammation can cause coronary artery aneurysms (CAAs). Patients with KD need cardiac assessment for risk stratification for the development of myocardial ischemia, based on Z-score (luminal diameter of the coronary artery corrected for body surface area). Echocardiography is the primary imaging modality in KD but has several important limitations. Coronary computed tomographic angiography (cCTA) and Cardiac MRI (CMR) are non-invasive imaging modalities and of additional value for assessment of CAAs with a high diagnostic yield. The objective of this single center, retrospective study is to explore the diagnostic potential of coronary artery assessment of cCTA vs. CMR in children with KD.Methods and Results: Out of 965 KD patients from our database, a total of 111 cCTAs (104 patients) and 311 CMR (225 patients) have been performed since 2010. For comparison, we identified 54 KD patients who had undergone both cCTA and CMR. CMR only identified eight patients with CAAs compared to 14 patients by cCTA. CMR missed 50% of the CAAs identified by cCTA.Conclusions: Our single center study demonstrates that cCTA may be a more sensitive diagnostic tool to detect CAAs in KD patients, compared to CMR.

Abstract 201: Risk Factors Of Persistent Coronary Artery Dilatation In Taiwanese Children With Kawasaki Disease.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Ming-Yu Liu ◽  
Hsin-Min Liu ◽  
Ming-Tai Lin ◽  
Chun-An Chen ◽  
Shuenn-Nan Chiu ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
Laboratory Data ◽  
Disease Onset ◽  
Z Score ◽  
Fever Onset ◽  
Coronary Artery Dilatation ◽  
Coronary Abnormalities ◽  
Over Time

Background: Kawasaki disease (KD) is an acute, systemic vasculitis disease of childhood, which may lead to cardiovascular complications, particularly coronary artery (CA) dilatation or aneurysm formation, and could result in morbidity and mortality. The Z score of coronary artery decreased from initial value within first few 2-3 months after fever onset. We follow the echocardiographic measurements of KD patients over time, and attempt to find the associated factors of persistent dilated coronary artery. Methods: Initial presentations, clinical laboratory data, echocardiography measurements and treatment were obtained from the patients with acute Kawasaki disease over 4 years period in a single medical center hospital. The patients were divided into 3 groups according to the initial maximum Z score of any coronary artery which were normalized for body surface area. We followed the echocardiography regularly at initial acute phase, 2-4 week, 5-12week, and > 3 months after fever onset. The maximal Z score of any coronary artery branches > +2 at any time were defined as having abnormalities. Results: We included total 169 patients with acute KD during 2008-2012. A maximal Z score for any of the coronary artery branches greater than +2 at acute phase was noted in 31.4% (53 of 169) of patients. During the following-up period, all except one patients (1 of 138) of the patients with initial maximal Z score <+2.5, the coronary artery have no abnormality at the end of following up. In contrast, the patients with initial maximal Z score≧+2.5 were more likely to have persistent coronary abnormalities over time (5 of 31, P<0.001). We also found hypoalbuminemia (P=0.006) and unresponsiveness to initial intravenous immunoglobulin treatment (P<0.001) associated with deteriorated or persistent CA abnormality within one month of disease onset. Conclusion: Coronary artery dilatation with Z score≧+2.5 at acute phase of Kawasaki disease, hypoalbuminemia and IVIG unresponsiveness are significantly associated with persistent CA abnormality at one month after KD onset. That indicated how to avoid IVIG unresponsiveness at the initial treatment of KD is a critical issue.

Abstract 222: North American Kawasaki Disease Registry - Advancing Clinical Research Through Collaboration

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Brian W McCrindle ◽  
Keyword(s):  
Kawasaki Disease ◽  
Clinical Research ◽  
North American ◽  
Data Entry ◽  
Practice Variation ◽  
Disease Registry ◽  
Funding Sources ◽  
The North ◽  
Number Of Patients ◽  
Sick Children

Background: Clinical research in children with Kawasaki disease (KD), particularly those with coronary artery aneurysms (CAA), is challenging due to the limited number of patients available at any single institution. This has resulted in imperfect evidence for optimal management and considerable practice variation. Methods: The North American Kawasaki Disease Registry (NAKDR) was started in July 2013 to determine the prevalence, patient-level and pharmacological risk factors for outcomes of CAA after KD. The NAKDR enrolls KD patients diagnosed from 1999-2013 with CAA (defined as any segment with a z-score >2.5). The NAKDR and its internet-based data entry portal are maintained at The Hospital for Sick Children in Toronto. Local ethics approvals and bilateral data sharing agreements are necessary for participation. Participation in the NAKDR is currently unfunded and voluntary. In September 2014, a survey on the future of the NAKDR was sent to all participating centers (response rate: 54%). Results: 45 sites have been invited, of which 37 (82%) agreed to participate. As of September 2014, 20 sites are actively submitting data; 17 are still being initiated; 706 cases have been submitted. The majority (90%) of members indicated that they wished to continue enrolling newly diagnosed patients and continue follow-up on patients already enrolled. Members were split (45% for, 55% against) as to whether the NAKDR should be expanded to include all KD patients, regardless of CAA status. Finally, while most NAKDR members (60%) indicated that site reimbursement was not an absolute condition for future participation, most members suggested that potential funding sources should be sought to expand/facilitate activities. At term, the NAKDR is expected to comprise ~1,400 patients and, if moving forward, add ~120 new KD patients with CAA per year. Conclusions: The NAKDR is an important tool for clinical research in children with CAAs after KD, and its success represents broad support from clinicians. The next step will be to formalize the NAKDR leadership structure, create standard operating procedures, and pursue prospective studies and funding.

scholarly journals Rare Refractory Kawasaki Disease in an Adolescent Boy With Cardiac and Diffuse Coronary Artery Involvement

Medicina ◽  
2013 ◽  
Vol 49 (7) ◽  
pp. 53
Author(s):  
Rima Šileikienė ◽  
Jolanta Kudzytė ◽  
Antanas Jankauskas ◽  
Liutauras Labanauskas ◽  
Vilma Rakauskienė ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Elderly Age ◽  
Coronary Artery Aneurysms ◽  
Cardiovascular Damage ◽  
Coronary Artery Involvement ◽  
Increased Risk ◽  
Adolescents And Adults ◽  
Appropriate Therapy ◽  
Infants And Young Children

Kawasaki disease is an acute multisystemic vasculitis occurring predominantly in infants and young children and rarely in adolescents and adults. At elderly age, Kawasaki disease may remain unrecognized with a subsequent delay in appropriate therapy and an increased risk of coronary artery aneurysms. We report a case of intravenous immunoglobulin- and aspirin-resistant Kawasaki disease and severe cardiovascular damage in an adolescent boy. The article discusses major issues associated with the management of refractory Kawasaki disease.

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