Outcomes of Kawasaki disease with giant coronary aneurysms: a single-centre study in southwest China

AbstractBackground:Giant coronary aneurysms are the most severe complications of Kawasaki disease. There are few reports of outcomes from China. Most previous studies were based only on absolute aneurysmal dimensions. The aim of the present study was to catalog the outcomes of Kawasaki disease with giant coronary aneurysms in southwest China based on absolute dimensions and the z-score adjusted for body surface area.Methods and results:All patients diagnosed with giant coronary aneurysms (z-score ≥ 10 or absolute dimension ≥ 8 mm) between December, 2002 and December, 2018 were included. We retrospectively analysed patient characteristics and clinical data from 38 patients with giant coronary aneurysms. Over a median follow-up period of 30.5 months (range from 1.7 months to 22.3 years), including patients in chronic phase who had been diagnosed prior to 2002, eight patients had myocardial infarction, including two deaths and one patient with coronary artery bypass grafting. The 1-, 2-, and 5-year event-free rates were 0.63, 0.63, and 0.53 for thrombosis, respectively, and 0.86, 0.81, and 0.81 for major adverse cardiac events, respectively. The 1-, 2-, and 5-year regression-free rates were 0.94, 0.85, and 0.67, respectively. A total of 73.7% of patients remained active.Conclusion:In the early stages of Kawasaki disease, patients with giant coronary aneurysms often experience major cardiovascular events; however, they are also likely to have normalisation of the coronary internal luminal diameter. With long-term anticoagulation, close cardiologic monitoring, and prompt thrombolytic therapy, most patients can achieve disease-free periods.

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Cardiac Events in Kawasaki Disease With Coronary Aneurysms Revisited, A Two-center Retrospective Cohort Study in Thailand

10.21203/rs.3.rs-252578/v1 ◽

2021 ◽

Author(s):

Kanokvalee Santimahakullert ◽

Chodchanok Vijarnsorn ◽

Yuttapong Wongswadiwat ◽

Prakul Chanthong ◽

Sappaya Khrongsrattha ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Artery Bypass ◽

Coronary Intervention ◽

Ivig Treatment ◽

Cardiac Events ◽

Coronary Aneurysms ◽

Adverse Cardiac Events ◽

Percutaneous Coronary

Abstract Kawasaki disease (KD) is a common vasculitis in children, which may be complicated with coronary artery aneurysms (CAAs). We aimed to report the rates of major adverse cardiac events (MACE) and determine the risks of MACE in children diagnosed with KD and CAAs in Thailand. Data of 170 children diagnosed with KD and CAAs in two centers of Thailand between 1994 and 2019 was retrospectively reviewed. The risks of MACE were analyzed using multivariate analysis. Of 170 patients, forty-nine patients (28.8%) had giant CAAs. During the median time of follow-up (5.4 years; ranging from 22 days to 23 years), 19 patients (11.1%) experienced MACE including 12 coronary artery bypass grafting, 2 percutaneous coronary intervention and 5 patients with evidence of coronary occlusion. Coronary interventions were performed at 4 years (ranging from 0.01 to 9.5 years) after the KD diagnosis. Independent risks of MACE in KD with CAAs were from the presence of giant aneurysms (HR 16.55; 95% CI 2.52 to 108.63; p=0.003) and lack of intravenous immunoglobulin (IVIG) treatment (HR 11.43; 95% CI 2.8 to 46.62; p=0.001). The intervention-free rate at 5 and 10 years in patients with giant CAAs was 78.7% and 52.2%, respectively.Trial registration: TCTR20190125004

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Abstract 212: Acute Myocardial Infarction in the Acute Phase in Kawasaki Disease in Mexican Children.

Circulation ◽

10.1161/circ.131.suppl_2.212 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

William Sarmiento-Robles ◽

Luis M Garrido-Garcia

Keyword(s):

Myocardial Infarction ◽

Kawasaki Disease ◽

Acute Phase ◽

Delayed Diagnosis ◽

Clinical Manifestations ◽

Unknown Origin ◽

Z Score ◽

Laboratory Parameters ◽

Small Series ◽

Coronary Aneurysms

Background: Kawasaki disease (KD) is an acute febrile vasculitis of unknown origin. Despite treatment with intravenous immunoglobulin during the acute phase of the disease, up to 5% of those affected will develop coronary aneurysms predisposing them to thrombotic complications that could result in myocardial infarction (AMI). In Mexico there are few reports of ischemic complications secondary to KD. Objective: To describe the clinical features, the laboratory parameters, treatment used and the outcome of children who presented with myocardial infarction during the acute phase of KD in a third level facility in Mexico City Methods: From our Institutional Database of KD we search for children who presented AMI in the acute phase of the disease from August 1995 to August 2014. We analyzed gender, age, clinical manifestations, time from the onset of the symptoms to diagnosis, laboratory parameters, treatment used, and outcome in the acute phase of the disease. Results: Eight infants were diagnosed with AMI during the study period. The median age at diagnosis was 8 months (range 2 to 53 months). Seven patients were male (87.5%). The median from the onset of the clinical manifestations to diagnosis of KD was 22 days (range 4 to 26 days). All patients developed giant coronary aneurysms (median Z-score 18.98, with a range of Z-score from 11.58 - 27.70). An abnormal EKG and abnormal perfusion tests demonstrated the myocardial infarction in all cases. Two patients died in the acute phase of cardiogenic shock, one more patient died of dilated cardiomyopathy 12 months after coronary bypass surgery with an overall mortality of 62.5% of this group. Conclusions: AMI is a fatal complication of KD. In our small series it was associated with a delayed diagnosis of the disease and therefore the development of giant coronary aneurysms. Treatment of AMI in children after KD is a medical challenge with a poor prognosis in children.

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Abstract 219: Long - Term Prognosis Of Patients With Kawasaki Disease Complicated By Significant Coronary Aneurysm (diameter ≥ 6 mm)

Circulation ◽

10.1161/circ.131.suppl_2.219 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Ji Seok Bang ◽

Gi Beom Kim ◽

Bo Sang Kwon ◽

Eun Jung Bae ◽

Chung Il Noh ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Coronary Artery Bypass ◽

Artery Bypass ◽

Coronary Aneurysm ◽

Coronary Intervention ◽

Surgical Interventions ◽

Coronary Aneurysms ◽

The Mean

Background: Some patients with Kawasaki disease (KD) develop large coronary aneurysms (diameter ≥ 6mm) and coronary stenosis, leading to ischemic heart disease. This study determined the long-term outcome for patients with Kawasaki disease complicated by significant coronary aneurysm. Methods and Results: From the database in Seoul National University Children’s Hospital between December 1986 and December 2013, medical records of 83 patients (61 men and 22 women) with large coronary aneurysms (diameter ≥ 6mm) were retrospectively reviewed. Information on patient demographics, catheter or surgical interventions, and most recent status was collected. From these data, we calculated the survival rate, cumulative coronary intervention rate, coronary artery bypass graft surgery rate. The mean age at onset was 4.28 ± 2.62 years, and the mean observational period was 13.2 ± 6.5 years. The maximum coronary artery internal diameter ranged from 6.1 to 25mm (median 9mm). Giant coronary aneurysm was 57 patients (68.7%) and large coronary aneurysm (6-8mm) was 26 patients (31.3%). Coronary aneurysms had progressed to coronary artery stenosis and/or complete occlusion in 42 patients (50.6%). The overall freedom from coronary intervention, coronary artery bypass surgery, or coronary thrombi was respectively 85.5%, 85.5%, and 90.3%. Catheter and surgical coronary interventions (median 1 intervention; range 1 to 5 interventions) were performed in 20 patients (24.1%) at 9 month to 18 years after onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8mm vs ≥8mm). Myocardial infarction occurred in 8 (8.98 %) patients. During this study period, 1 patient died and 98% survival rates was seen in our patients group. Conclusion: The long-term survival of patients with Kawasaki disease complicated by large coronary aneurysm is good even though a fourth of patients underwent multiple catheter or surgical interventions. Further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions in large coronary aneurysm (≥ 6mm).

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

Children ◽

10.3390/children5110155 ◽

2018 ◽

Vol 5 (11) ◽

pp. 155 ◽

Cited By ~ 1

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.

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

Circulation ◽

10.1161/circ.131.suppl_2.o58 ◽

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.

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Cardiac events in Patients in their forties with Kawasaki disease and regression of coronary artery aneurysms

Cardiology in the Young ◽

10.1017/s104795112000284x ◽

2020 ◽

Vol 30 (12) ◽

pp. 1821-1825 ◽

Cited By ~ 1

Author(s):

Etsuko Tsuda ◽

Shuichi Yoneda ◽

Yasuhide Asaumi ◽

Atsuko Suzuki

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Coronary Arteries ◽

Coronary Artery Calcification ◽

Coronary Aneurysm ◽

Coronary Artery Lesions ◽

Cardiac Events ◽

Coronary Artery Aneurysms ◽

Coronary Aneurysms ◽

Middle Aged Adult

AbstractOver a 50-year period from the first description of Kawasaki disease, we encountered three male patients with a history of Kawasaki disease, who had their first cardiac events in their forties. They were considered to have almost normal coronary arteries in the coronary angiograms when they were children and adolescents. They had no follow-up examinations after 20 years old. The 1st patient had an acute myocardial infarction, and the 2nd was a new appearance of coronary aneurysm and stenotic lesions with coronary artery calcification. The 3rd patient had unexpected sudden death. The interval from the onset of Kawasaki disease to the cardiac events ranged from 37 to 38 years. In the former two patients, coronary artery lesions could not be evaluated immediately after Kawasaki disease. Although the 3rd patient had bilateral medium-sized coronary artery aneurysms, his coronary aneurysms regressed 1 year after acute Kawasaki disease. The intimal thickening at a previous coronary aneurysm at the age of 19 was mild. The patients with regressed coronary aneurysms were asymptomatic for about 40 years after Kawasaki disease, prior to their cardiac events. Coronary artery calcification of the proximal portion of the major coronary arteries was a predictable marker in such patients. To prevent serious cardiac events in middle-aged adult patients, reevaluation of coronary artery lesions and restarting of anti-thrombotic therapy are needed. We must be aware that there are some differences in the clinical course and time of cardiac events between patients with giant aneurysms and those with medium aneurysms.

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Abstract 13850: Severity Assessment of Coronary Artery Aneurysm by Z-score of the Internal Diameter in Kawasaki Disease

Circulation ◽

10.1161/circ.132.suppl_3.13850 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Masaru Miura ◽

Naoya Fukushima ◽

Tetsuji Kaneko ◽

Tohru Kobayashi ◽

Kenji Suda ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Cox Regression ◽

The Body ◽

Time Dependent ◽

Occurrence Rate ◽

Severity Assessment ◽

Z Score ◽

Cardiac Events ◽

Coronary Events

Introduction: The severity of coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) has been recently classified according to the z-score. However, it is not known whether this classification can predict coronary events such as stenosis, obstruction, and thrombosis. Methods: In this multicenter retrospective study, data on height, weight, CAA diameter measured by echocardiography in the acute phase, and the clinical course in KD patients 18 years of age or younger who received a coronary angiography between 1992 and 2011 were collected. Time-dependent occurrence of coronary events was analyzed by Kaplan-Meier method according to small (z-score, < 5.0), medium (≥ 5.0 to < 10.0), and large (≥ 10.0) CAA using a 5 increment scale scheduled to be included in the new American Heart Association criteria. Cox regression analysis was used to identify risk factors for coronary events. The occurrence rate of major cardiac events such as angina pectoris, myocardial infarction, and cardiac death was also analyzed. Results: Data were analyzed for 1,002 patients from 44 institutions. Both the body surface area and CAA diameters were available in 741 cases for the right coronary artery (RCA) and 609 cases for the left anterior descending artery (LAD). Coronary events occurred in 83 (11.2%) of the RCA group and 57 (9.4%) of the LAD group, while major events occurred in 30 cases (3.0%). The 10-year event-free survival rate for coronary events for small, medium, and large aneurysms was 100, 95.5, and 64.9% in the RCA group, and 100, 94.4, and 63.5% for aneurysms in the LAD group, respectively. The rate of major cardiac events was 98.5, 98.1, and 87.6% for the RCA group, and 100, 97.5, and 86.8% for the LAD group, respectively. Cox regression analyses showed that the z-score of the CAA diameter was an independent risk factor for coronary events for the RCA [large versus medium aneurysm; hazard ratio (HR) 2.8, 95% confidence interval (CI) 1.5 to 5.3, p = 0.002] and the LAD [HR 3.2, 95% CI 1.6 to 6.5, p = 0.015] groups. Conclusions: The severity assessment of CAA using the 5-increment z-score for coronary arterial diameter can predict the time-dependent occurrence of coronary events in patients with KD.

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Non-coronary cardiac events, younger age, and IVIG unresponsiveness increase the risk for coronary aneurysms in Italian children with Kawasaki disease

Clinical Rheumatology ◽

10.1007/s10067-020-05331-w ◽

2020 ◽

Author(s):

Marianna Fabi ◽

Laura Andreozzi ◽

Ilaria Frabboni ◽

Ada Dormi ◽

Elena Corinaldesi ◽

...

Keyword(s):

Kawasaki Disease ◽

Cardiac Events ◽

Coronary Aneurysms ◽

Younger Age ◽

Italian Children

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Variation in the management of Kawasaki disease

Archives of Disease in Childhood ◽

10.1136/archdischild-2019-317191 ◽

2019 ◽

Vol 105 (10) ◽

pp. 1004-1006 ◽

Cited By ~ 2

Author(s):

Audrey Dionne ◽

David Burgner ◽

Sarah De Ferranti ◽

Davinder Singh-Grewal ◽

Jane Newburger ◽

...

Keyword(s):

Kawasaki Disease ◽

Initial Treatment ◽

Coronary Aneurysm ◽

Medical Specialty ◽

Primary Treatment ◽

Management Options ◽

Necrosis Factor Alpha ◽

Coronary Aneurysms ◽

Factor Alpha ◽

Collaborative Efforts

Intravenous immunoglobulin (IVIG) reduces coronary aneurysms in patients with Kawasaki disease (KD), but additional management options remain challenging, with no generalisable evidence-based recommendations. We performed a survey of 724 physicians from 73 countries to assess variation in practice. IVIG was the preferred initial treatment by 659 (91%) of respondents. Criteria for adjunctive primary treatment varied considerably and definitions of IVIG resistance varied markedly by geographical continent, Human Development Index tiers and medical specialty. A second dose of IVIG was used most often for patients with coronary aneurysm non-responsive to initial treatment (572, 79%), but corticosteroids (379, 52%) and tumour necrosis factor alpha inhibitors (208, 29%) were also frequently used. Our findings highlight the need for international collaborative efforts to optimise management of patients with KD worldwide.

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