Abstract O.37: Coronary Artery Dilatation in Viral Myocarditis Mimics Coronary Artery Findings In Kawasaki Disease

Background: Coronary artery dilatations are almost always secondary to Kawasaki disease in the pediatric population. The presence of CA involvement is used as a criterion to diagnose incomplete KD disease, which may be challenging. It has been recently demonstrated that febrile patients had larger CA size than non-febrile children in a pilot study. As there is almost always a myocarditis in the acute phase of KD disease we sought to investigate whether viral myocarditis may cause CA dilatations. Method: This retrospective study reviewed 14 consecutive patients with a diagnosis of viral myocarditis at Saint-Justine Hospital, Montreal, from April 2000 trough December 2010. Kawasaki disease was excluded in all patients. All echocardiogram studies were reviewed by an independent experienced technician for CA size and function parameters. Patients were classified in three categories: definite CA dilatation (Z-score ≥ 2.5 in one or more CA), occult CA dilatation (Z score variation ≥ 2 for the same CA on 2 different echogrardiograms, but absolute Z score always < 2.5) and normal coronary artery. Demographics, laboratory values, microbial etiology testing, diagnostic studies were also collected. Results: Mean age at presentation was 1.67 ± 3.22 years, the majority < 2 years old, and 11 (78%) were girls. Of the 14 patients 8 (57.1%) had normal CA, 3 (21.4%) had occult CA dilatation and 3 (21.4%) had definite CA dilatation. When present, CA dilatation was detected within the first 8 days of presentation. Eleven (78%) patients presented with acute onset features and the remaining 3 presented with subacute characteristics. There was no correlation between CA involvement and the intensity of LV dysfunction however (p = 0.84). Conclusion: Patients with viral myocarditis can present CA dilatation during the acute phase of the illness. This finding should be taken into account when diagnosing patients with incomplete KD on the basis of the CA involvement as the two illnesses may present with similar features.

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

Circulation ◽

10.1161/circ.131.suppl_2.201 ◽

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.

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Abstract 123: Long term Prognosis After Methylprednisolon pulse Combination Therapy For Refractory Kawasaki Disease

Circulation ◽

10.1161/circ.131.suppl_2.123 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Takasuke Ebato ◽

Yoshihito Ogihara ◽

Manabu Takanashi ◽

Takashi Honda ◽

Sumito Kimura ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Combination Therapy ◽

Adverse Events ◽

Acute Phase ◽

University Hospital ◽

Recurrent Fever ◽

Z Score ◽

Long Term Prognosis

Background: We previously investigated the molecular mechanism and the clinical utility of intravenous methylprednisolone-pulse(IVMP) plus intravenous immunoglobulin (IVIG) combination therapy (IVMP+IVIG) for patients with Kawasaki disease (KD) in the acute phase (Pediatr Res 2009, Pediatrics 2012). However, the long-term prognosis after IVMP+IVIG still remains unclear. Objective: To examine the long-term prognosis after IVIG+IVMP. Methods: Forty-six patients (Age: 5-70 months, male: 27) who were enrolled in this study were treated with IVMP+IVIG during the period from 2007 to 2014 at Kitasato University Hospital who were all predicted to show resistance to initial IVIG according to their Egami score (J Pediatr 2006). The IVMP therapy (30mg/kg, 1dose) was administered for 2 hours before starting the IVIG therapy. Heparin (10U/kg/h, continuous infusion) was used concomitantly from 2 hours before the start of IVMP therapy and it was thereafter continued for 24 hours. The long-term prognosis of coronary artery lesions (CALs) and adverse events between the time of diagnosis and 5 years after IVMP +IVIG were determined. The “Z-score2.5-5” and “Z–score>5” were defined as dilatation and an aneurysm, respectively, according to the AHA guidelines. Results: Dilatation cases based on the Z-score2.5-5, were identified in 15 of 46 patients (32.6%) at diagnosis, in 15 of 46 patients (32.6%) after 1 month, and in 3 of 36 patients (8.3%) after 1 year. All patients in whom coronary artery abnormalities were observed at 1 year later demonstrated regression.No patients showed a Z-score≧5 in all phases. Thirty-six patients (78.2%) showed prompt defervescence owing to the combination therapy. The patients had adverse event at acute phase, 16 of 46 patients (34.7%) for hypothermia, 5 patients (10.8%) for hypertension, 2 patients (4.3%) for bradycardia, and 4 patients (8.6%) for recurrent fever. No patients experienced any serious adverse events (short stature, hyperlipidemia, abnormal glucose tolerance) after IVMP +IVIG during the long-term follow-up. Conclusions: This study showed IVMP+IVIG combination therapy to therefore effectively prevent CALs in KD patients that are predicted to be resistant to intial IVIG according to their Egami score.

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Abstract O.68: Corticosteroid Pulse Therapy for Acute Kawasaki Disease: Consideration for the Long-Term Prognosis of Coronary Artery Lesions

Circulation ◽

10.1161/circ.131.suppl_2.o68 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Atsushi Kitagawa ◽

Masahiro Ishii ◽

Yoshihito Ogihara ◽

Shouhei Ogata ◽

Motofumi Iemura ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Pulse Therapy ◽

Z Score ◽

Coronary Artery Lesions ◽

Corticosteroid Administration ◽

Corticosteroid Pulse Therapy ◽

Long Term Prognosis

Background: The use of corticosteroids as treatment for Kawasaki disease (KD) patients is still controversial. And the effects of corticosteroids on coronary artery lesions (CALs) development and later vascular remodeling are also unclear. The purpose of this study was to compare the long term prognosis of KD with CALs between corticosteroid administration patients and no corticosteroid using patients. Methods: Five hundred sixty nine patients with KD were studied at Kurume University Hospital from 1996 to 2004. Clinical records of 66 patients (46 males, 20 females) with CALs were reviewed. The median age at diagnosis was 1.5 (range 0.2 - 13.2) years and median follow-up period was 8.9 (range 0.1 - 16.4) years. Coronary artery sizes were measured by body surface area (BSA) adjusted z-score to using echocardiography. CALs were defined as coronary artery z-score > 2.5, and CAL regressions were defined as z-score < 2.5. Results: Sixty four patients were treated with intravenous immunoglobulin (IVIG), and 51 (79.7%) patients were unresponsive to the initial IVIG treatment. Twenty seven (40.9%) patients were received corticosteroid pulse therapy in the acute phase. The maximum CAL z-score in the acute phase, there were not significant differences between corticosteroid administration patients and no corticosteroid using patients (5.1 ± 2.2 vs. 4.9 ± 2.3, p = 0.277). The CAL z-score at the end of this study period, there were not significant differences between two groups (2.1 ± 2.0 vs. 2.3 ± 2.3, p = 0.432). The ratio of CAL regression in the study period (33.3% vs. 46.2%), the mean interval between the onset of KD and CAA regression (0.6 ± 0.5 vs. 0.8 ± 0.5 years, p = 0.209), and the ratio of coronary artery stenosis or occlusion (14.8% vs. 15.4%), there were not significant differences between two groups. Conclusion: Corticosteroid pulse therapy for KD patients may not be worsened CALs in the acute phase and long-term after the onset.

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Z-score is a possible predictor of the risk of coronary artery lesion development in patients with Kawasaki disease in Japan

European Journal of Pediatrics ◽

10.1007/s00431-021-04006-1 ◽

2021 ◽

Author(s):

Takayuki Suzuki ◽

Nobuyuki Kakimoto ◽

Tomoya Tsuchihashi ◽

Tomohiro Suenaga ◽

Takashi Takeuchi ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Coronary Artery Lesion ◽

Z Score ◽

Lesion Development

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0328: Evaluation of coronary artery wall echogenicity in Kawasaki disease acute phase

Archives of Cardiovascular Diseases Supplements ◽

10.1016/s1878-6480(15)71782-9 ◽

2015 ◽

Vol 7 (1) ◽

pp. 102

Author(s):

Fanny Riou ◽

Aurélie Chalard ◽

Aurélie Chausset ◽

Jean René Lusson ◽

Etienne Merlin ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Artery Wall ◽

Coronary Artery Wall

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Evaluation of coronary artery wall echogenicity in Kawasaki disease acute phase

Archives of Cardiovascular Diseases ◽

10.1016/j.acvd.2014.07.004 ◽

2014 ◽

Vol 107 (8-9) ◽

pp. 485

Author(s):

F. Riou ◽

A. Chalard ◽

A. Chausset ◽

J.R. Lusson ◽

E. Merlin ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Artery Wall ◽

Coronary Artery Wall

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

Circulation ◽

10.1161/circ.131.suppl_2.221 ◽

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.

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Atypical coronary artery aneurysms due to Kawasaki disease in Noonan syndrome with a novel PTPN11 mutation

Cardiology in the Young ◽

10.1017/s1047951118001877 ◽

2018 ◽

Vol 29 (2) ◽

pp. 228-230

Author(s):

Shiori Takai ◽

Kei Takasawa ◽

Shozaburo Doi

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Therapeutic Intervention ◽

Noonan Syndrome ◽

Coronary Artery Aneurysms ◽

Ptpn11 Mutation

AbstractWe report a 3-year-old boy with giant and atypical coronary artery aneurysms in the acute phase of Kawasaki disease, despite appropriate therapeutic intervention, in Noonan syndrome with a novel heterozygous PTPN11 mutation, c. 907 G>A (p.Asp303Asn). We hypothesised that this PTPN11 mutation might affect both hyperinflammation caused by Kawasaki disease and vascular fragility in the coronary artery, resulting in coronary artery aneurysms.

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Abstract 28: African American Race is Not Protective Against Coronary Artery Involvement in Kawasaki Disease

Circulation ◽

10.1161/circ.131.suppl_2.28 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Emily P Williams ◽

Michael S Kelleman ◽

William T Mahle

Keyword(s):

African American ◽

Coronary Artery ◽

Kawasaki Disease ◽

Reference Group ◽

Artery Aneurysm ◽

Z Score ◽

Strict Adherence ◽

Hispanic Patients ◽

American Children ◽

African American Race

It has been previously reported that African American race may be protective against coronary artery aneurysm development in Kawasaki Disease (KD). We aimed to test this with our own cohort of KD patients from a large pediatric cardiology practice. Data from 250 subjects diagnosed with KD and followed as outpatients with surveillance echocardiography over a two-year period were analyzed. Twelve patients were excluded due to incomplete records or an unconfirmed diagnosis. Race designated by parent was recorded. Charts were reviewed for any coronary involvement (ectasia or aneurysm) and coronary Z-score greater than 2.5 at the time of diagnosis and at subsequent follow-up visits. Odds rations were calculated comparing each racial group to others for any coronary involvement and for coronary Z-score > 2.5. Of 238 included patients, 44.5% were African American, 37.4% were non-Hispanic white, 10.5% were Hispanic, and 7.6% identified with other racial designations. Approximately 21.9% of African American patients had any coronary involvement and 9.5% had a coronary Z-score > 2.5. Approximately 21.4% of non-Hispanic whites had any coronary involvement and 13.5% of non-Hispanic whites had a coronary Z-score > 2.5. Twenty-eight percent of Hispanic patients had any coronary involvement and 12% had a coronary Z-score > 2.5%. Of patients that identified with other racial designations, 38.9% had coronary involvement and 22.2% had a coronary Z-score > 2.5. No statistically significant odds ratios were identified. Relative to reference group (non-Hispanic whites) African American patients had nearly identical rates of 1) any coronary involvement, or 2) coronary Z-score > 2.5. KD occurs commonly in African-American children. Given equal risk for late coronary sequelae vigilance and strict adherence to consensus guidelines is essential.

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