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

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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Predictors for coronary artery dilatation in Kawasaki disease

Paediatrica Indonesiana ◽

10.14238/pi58.5.2018.257-62 ◽

2018 ◽

Vol 58 (5) ◽

pp. 257-62

Author(s):

Najib Advani ◽

Anisa Rahmadhany ◽

Sarah Rafika

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Predictive Factors ◽

Laboratory Data ◽

Febrile Illness ◽

Heart Association ◽

Inclusion Criteria ◽

Cross Sectional ◽

Coronary Artery Dilatation

Background Kawasaki disease (KD) is an acute, self-limited, febrile illness of unknown cause that predominantly affects children below 5 years of age. It has a high incidence of coronary complications such as aneurysms. The current treatment of choice is intravenous immunoglobulin, which is costly, with aspirin. Identifying the predictive factors for coronary artery dilatation or aneurysm is important in order to establish the indications for giving immunoglobulin, especially when resources are limited. Objective To identify the predictors for the development of coronary artery dilatation in patients with Kawasaki disease Methods This cross-sectional study was done between January 2003 and July 2013. Inclusion criteria were patients who fulfilled the American Heart Association criteria for acute Kawasaki disease, and had complete clinical, echocardiogram, and laboratory data [hemoglobin, leukocyte, platelet, albumin, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR)]. All of them received immunoglobulin and aspirin. Results Of 667 KD patients, 275 met the inclusion criteria. There were 185 (67%) males. Subjects’ ages varied between 1 to 157 months. The frequency of coronary artery dilatation at the acute phase was 33.3%. Multivariate analysis showed that >7-day duration of fever and hypoalbuminemia were significant predictive factors for coronary artery dilatation. Conclusion Predictive factors for coronary artery dilatation are duration of fever over 7 days and hypoalbuminemia, while age, gender, hemoglobin level, leukocyte count, and platelet count are not. Frequency of coronary artery dilatation at the acute phase is 33.3%.

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

Circulation ◽

10.1161/circ.131.suppl_2.o37 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Soha Rached-d'Astous ◽

Ibtissama Boukas ◽

Anne Fournier ◽

Marie-Josée Raboisson ◽

Nagib Dahdah

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Pediatric Population ◽

Viral Myocarditis ◽

Acute Onset ◽

Z Score ◽

Lv Dysfunction ◽

Microbial Etiology ◽

And Function

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 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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N-terminal pro-brain natriuretic peptide in acute Kawasaki disease correlates with coronary artery involvement

Cardiology in the Young ◽

10.1017/s1047951114002431 ◽

2014 ◽

Vol 25 (7) ◽

pp. 1311-1318 ◽

Cited By ~ 9

Author(s):

Philippe M. Adjagba ◽

Laurent Desjardins ◽

Anne Fournier ◽

Linda Spigelblatt ◽

Martine Montigny ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Brain Natriuretic Peptide ◽

Intravenous Immunoglobulin ◽

Natriuretic Peptide ◽

Brain Natriuretic Peptide Level ◽

Z Score ◽

Natriuretic Peptide Level ◽

Coronary Artery Dilatation ◽

Peptide Level

AbstractBackgroundWe have lately documented the importance of N-terminal pro-brain natriuretic peptide in aiding the diagnosis of Kawasaki disease.ObjectivesWe sought to investigate the potential value of N-terminal pro-brain natriuretic peptide pertaining to the prediction of coronary artery dilatation (Z-score>2.5) and/or of resistance to intravenous immunoglobulin therapy. We hypothesised that increased serum N-terminal pro-brain natriuretic peptide level correlates with increased coronary artery dilatation and/or resistance to intravenous immunoglobulin.MethodsWe carried out a prospective study involving newly diagnosed patients treated with 2 g/kg intravenous immunoglobulin within 5–10 days of onset of fever. Echocardiography was performed in all patients at onset, then weekly for 3 weeks, then at month 2, and month 3. Coronary arteries were measured at each visit, and coronary artery Z-score was calculated. All the patients had N-terminal pro-brain natriuretic peptide serum level measured at onset, and the Z-score calculated.ResultsThere were 109 patients enrolled at 6.58±2.82 days of fever, age 3.79±2.92 years. High N-terminal pro-brain natriuretic peptide level was associated with coronary artery dilatation at onset in 22.2 versus 5.6% for normal N-terminal pro-brain natriuretic peptide levels (odds ratio 4.8 [95% confidence interval 1.05–22.4]; p=0.031). This was predictive of cumulative coronary artery dilatation for the first 3 months (p=0.04–0.02), but not during convalescence at 2–3 months (odds ratio 1.28 [95% confidence interval 0.23–7.3]; p=non-significant). Elevated N-terminal pro-brain natriuretic peptide levels did not predict intravenous immunoglobulin resistance, 15.3 versus 13.5% (p=1).ConclusionElevated N-terminal pro-brain natriuretic peptide level correlates with acute coronary artery dilatation in treated Kawasaki disease, but not with intravenous immunoglobulin resistance.

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Abstract 147: Coronary artery aneurysms on day 4 after onset of Kawasaki Disease: A Case report

Circulation ◽

10.1161/circ.131.suppl_2.147 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Anzai Tatsuya ◽

Takaomi Minami ◽

Sadahiro Furui ◽

Kensuke Oka ◽

Akiko Yokomizo ◽

...

Keyword(s):

Case Report ◽

Coronary Artery ◽

Kawasaki Disease ◽

Laboratory Data ◽

Density Lipoprotein ◽

Artery Aneurysm ◽

Main Trunk ◽

Reactive Protein ◽

Coronary Artery Dilatation ◽

Old Japanese

Background: Coronary artery aneurysm (CAA) in patients with Kawasaki disease (KD) almost always occurs after day 8, and the most early detection report of CAA was in an autopsy case on day 6 after onset. The possibility of CAA formation prior to day 6 has not been documented. Case report: A 10-month-old Japanese boy presented with a 4-day fever, conjunctival injection, erythema of the lips, erythema of the palms and soles, erythematous rush, and induration at a Bacillus Calmette-Guerin inoculation site. On day 4, echocardiography revealed coronary artery dilatation (right coronary artery [RCA], 4.6 mm; left main trunk [LMT], 4.6 mm; left anterior descending [LAD] artery, 3.4 mm). Laboratory data were as follows: white blood cell count, 19,400/μL; C-reactive protein, 15.2 mg/dL; albumin, 3.2 g/dL; high-density lipoprotein cholesterol, 20 mg/dL, and erythrocyte sedimentation rate, 48 mm/h. He was diagnosed with KD and treated with oral aspirin 30 mg/kg/day and intravenous immunoglobulin (IVIG) 2 g/kg. His fever subsided the next day. On day 17, periungual peeling of the fingers and toes was noted. His fever recurred and he treated with additional IVIG 1 g/kg, after which his fever subsided again. On day 20, the aneurysms had not increased in size (RCA, 3.3 mm; LMT, 3.6 mm; LAD, 2.4 mm). On day 44, although echocardiography showed a mild coronary artery dilatation, a coronary angiogram showed no apparent abnormality. He had no obvious fever or symptoms of KD prior to this course of KD. This case may help clarify the pathophysiology of CAA in patients with KD.

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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

10.21203/rs.3.rs-226772/v1 ◽

2021 ◽

Author(s):

Takayuki Suzuki ◽

Nobuyuki Kakimoto ◽

Tomoya Tsuchihashi ◽

Tomohiro Suenaga ◽

Takashi Takeuchi ◽

...

Keyword(s):

Risk Factors ◽

Coronary Artery ◽

Kawasaki Disease ◽

Characteristic Curve ◽

Laboratory Data ◽

Delayed Diagnosis ◽

Area Under The Curve ◽

Coronary Artery Lesion ◽

Z Score ◽

Male Sex

Abstract ABSTRACT Risk factors for coronary artery lesion (CAL) development in patients with Kawasaki disease (KD) include male sex, age <12 months, intravenous immunoglobulin (IVIG) resistance, and delayed diagnosis. We aimed to explore the relationship between CAL development and Z-score. We enrolled 281 patients with KD who were treated with our protocol. Echocardiography was performed in three phases: pre-treatment (P1), post-treatment (P2), and 4 weeks after onset (P3). The highest Z-score of the right, left main, left anterior descending, and left circumflex coronary arteries was expressed as Zmax at each phase. P3-Zmax ≥2.5 represented CAL development. Clinical parameters, such as laboratory data and Z-scores, were retrospectively compared between patients with and without CAL development. Sixty-seven patients (23.8%) showed a P1-Zmax ≥2.0, and CAL development occurred in 21 patients (7.5%). Independent risk factors associated with CAL development were P1-Zmax, a ΔZmax (P2-Zmax − P1-Zmax) ≥1, male sex, <12 months of age, and resistant to the first IVIG administration (adjusted odds ratio [95% confidence interval]: 1.98 [1.01–3.92], 4.04 [1.11-14.7], 6.62 [1.33–33.04], 4.71 [1.51–14.68], 5.26 [1.62–17.13], respectively). Using receiver operating characteristic curve analysis, a P1-Zmax ≥1.43 detected CAL development with an area under the curve of 0.64 (sensitivity = 81.0%; specificity = 48.1%). Conclusions : Our results suggest that P1-Zmax and a ΔZmax (P2-Zmax − P1-Zmax) ≥1 may predict CAL development.

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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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Rare case of Kawasaki disease with cardiac tamponade and giant coronary artery aneurysms

Cardiology in the Young ◽

10.1017/s1047951120004989 ◽

2021 ◽

pp. 1-2

Author(s):

Andrea Vergara ◽

Emanuele Monda ◽

Cinzia Mautone ◽

Francesca Renon ◽

Antonio Di Masi ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Cardiac Tamponade ◽

Acute Phase ◽

Circumflex Coronary Artery ◽

Coronary Artery Aneurysms ◽

Fever Onset ◽

Cardiovascular Involvement ◽

Peculiar Case ◽

Proximal Right Coronary Artery

Abstract Kawasaki disease is an acute systemic vascular disease, generally self-limited and typically affecting children <5 years old, which leads to coronary artery aneurysms in about 25% of untreated cases. Cardiovascular involvement is characterised by transient pancarditis, in acute phase, while coronary illness, ranging from mild dilation to giant CAAs occurs late, rarely before the 10th day since fever onset. Here, we describe a peculiar case of KD, which occurred in a 4-month-old infant and presented with exudate cardiac tamponade and early giant aneurism of both the proximal right coronary artery) and the left circumflex coronary artery, in acute phase of the disease.

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Desquamation in Kawasaki Disease

Children ◽

10.3390/children8050317 ◽

2021 ◽

Vol 8 (5) ◽

pp. 317

Author(s):

Ling-Sai Chang ◽

Ken-Pen Weng ◽

Jia-Huei Yan ◽

Wan-Shan Lo ◽

Mindy Ming-Huey Guo ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Laboratory Data ◽

Ivig Treatment ◽

Low Grade ◽

High Grade ◽

Laboratory Findings ◽

Coronary Artery Abnormalities ◽

Cell Counts ◽

Hands And Feet

(1) Background: Desquamation is a common characteristic of Kawasaki disease (KD). In this study, we analyzed patients’ varying desquamation levels in their hands or feet, in correlation with clinical presentation, to assess the relationship. (2) Methods: We retrospectively reviewed children with KD. We analyzed their age, laboratory data before intravenous immunoglobulin (IVIG) treatment and coronary artery abnormalities (CAA) based on the desquamation level of their hands and feet. We classified the desquamation level from 0 to 3 and defined high-grade desquamation as grade 2 and 3. (3) Results: We enrolled a total 112 patients in the study. We found the hands’ high-grade desquamation was positively associated with age and segmented neutrophil percentage (p = 0.047 and 0.029, respectively) but negatively associated with lymphocyte and monocyte percentage (p = 0.03 and 0.006, respectively). Meanwhile, the feet’s high-grade desquamation was positively associated with total white blood cell counts (p = 0.033). Furthermore, we found that high-grade hand desquamation had less probability of CAA formation compared with that of a low grade (7.1% vs. 40.8%, p = 0.016). (4) Conclusions: This report is the first to demonstrate that the desquamation level of hands or feet in KD is associated with different coronary artery abnormalities and laboratory findings.

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