Outcome of coronary artery aneurysms after Kawasaki disease

doi:10.1016/S0022-3476(05)81894-3

The Journal of Pediatrics

Volume 121, Issue 5, Part 1, November 1992, Pages 689-694

https://doi.org/10.1016/S0022-3476(05)81894-3 Get rights and content

From 1974 through 1991, a total of 583 children with Kawasaki disease were seen at the Hospital for Sick Children, in Toronto, of whom 80 (13.7%) had coronary artery involvement. There were 55 boys and 25 girls, whose mean age at onset was 2.9±2.5 years, followed for a mean period of 4.0±3.6 years. Giant aneurysms (maximum diameter ≥8 mm) were found in 22 children, moderate-sized aneurysms (≥4 to <8 mm) in 44, and dilation lesions (<4 mm) in 14. Myocardial infarction occurred in 9 (1.5%), all of whom had giant aneurysms. The persistence rate for aneurysms was 72% at 1 year and 41% at 5 years of follow-up. In multivariate analysis, the regression of an aneurysm was significantly related to the severity of coronary artery lesions, initial treatment, and gender. Although >80% of small or moderate-sized aneurysms regressed within 5 years, giant aneurysms did not regress during the follow-up period. In patients who received immune globulin therapy, coronary lesions tended to resolve more rapidly than in those treated with salicylate therapy alone, because 91% of the lesions in the former were small or moderate. These findings suggest that the severity of coronary artery involvement during the initial stages of Kawasaki disease influences the regression of these lesions, and that immune globulin treatment may improve outcome by reducing the incidence of severe lesions.

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Cited by (195)

French national diagnostic and care protocol for Kawasaki disease
2023, Revue de Medecine Interne
Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.
La maladie de Kawasaki (MK) est une vascularite aiguë ayant un tropisme particulier pour les artères coronaires. La MK touche préférentiellement les enfants âgés de 6 mois à 5 ans, avec une prédominance masculine. Le diagnostic est clinique, basé sur les critères internationaux de l’American Heart Association, et doit être posé systématiquement chez les enfants présentant une fièvre depuis 5 jours ou plus, ou depuis 3 jours si tous les critères sont présents. Il est important de noter que la plupart des enfants présentent une irritabilité marquée et parfois des signes digestifs. Une inflammation biologique marquée, bien que non spécifique, est d’une grande valeur pour le diagnostic. En raison des difficultés à reconnaître la MK dans les formes incomplètes ou atypiques, et de l’urgence du traitement, l’enfant doit être référé à un hôpital pédiatrique dès que le diagnostic est simplement suspecté. En cas de signes d’insuffisance cardiaque (pâleur, tachycardie, polypnée, sueurs, hépatomégalie, instabilité tensionnelle), un transfert en soins intensifs est indispensable. Le traitement de référence repose sur les IgIV associée à l’aspirine, (avant 10 jours de fièvre, et pour une durée minimale de 6 semaines), ce qui réduit le risque d’anévrisme coronarien. En cas d’atteinte coronarienne, les antiagrégants peuvent être maintenus à vie. En cas d’anévrisme géant, les anticoagulants sont ajoutés aux antiagrégants. Le pronostic de la MK est généralement bon, la majorité des enfants se rétablissant sans séquelles. En cas d’atteinte coronarienne initiale, le pronostic dépend de l’évolution de l’atteinte cardiaque telle qu’elle est définie au cours d’un suivi cardiologique rapproché.
Incidence and timing of coronary thrombosis in Kawasaki disease patients with giant coronary artery aneurysm
2023, Thrombosis Research
Coronary thrombosis is a common cardiovascular complication of Kawasaki disease (KD), which seriously affects the long-term therapeutic effect of KD. The purpose was to determine the incidence and timing of coronary thrombosis and to identify risk factors for coronary thrombosis in KD with giant coronary artery aneurysm (GCAA).
A total of 94 consecutive KD patients with GCAA from Children's Hospital Affiliated to Chongqing Medical University were enrolled retrospectively. The cumulative incidence of coronary thrombosis in KD patients with GCAA was 59 % (n = 54). Coronary thrombosis mainly occurred in the acute phase (n = 41/54, 76 %), with a median time of 16 days after onset. Cox regression analysis was used to identify risk factors for coronary thrombosis. Cox regression analysis indicated that male (hazard ratios, 1.87; 95 % CI, 1.01–3.44; P = 0.43), left anterior descending artery (LAD) involvement (hazard ratios, 3.75; 95 % CI, 1.85–7.39; P < 0.001), coronary absolute diameter ≥ 8 mm (hazard ratios, 2.93; 95 % CI, 1.36–6.29; P = 0.006) constituted a higher risk of coronary thrombosis after adjusting for confounders. Kaplan-Meier method showed the cumulative incidence for coronary thrombosis in KD patients with GCAA was 79 %, 92 %, and 88 % in male, LAD involvement, coronary absolute diameter > 8 mm, respectively.
Male, LAD involvement, and coronary absolute diameter ≥ 8 mm were associated with a high incidence of coronary thrombosis. Based on the analysis of the incidence, time and risk factors of coronary thrombosis in different periods, this study may provide an essential reference for thromboprophylaxis management of KD with GCAA.
Epidemiology, Treatments, and Cardiac Complications in Patients with Kawasaki Disease: The Nationwide Survey in Japan, 2017-2018
2020, Journal of Pediatrics
Citation Excerpt :
A previous report estimated that the actual number of patients with Kawasaki disease in Japan might be ≤10% higher than the number recorded in the survey.10 Second, the survey noted CAAs mostly based on the Japanese criteria.14,15 However, z-score evaluations, the criteria used worldwide, could increase the recognition of CAAs compared with the Japanese criteria.21,43,44
To report the epidemiologic characteristics, treatments, and cardiac complications of Kawasaki disease, using data from the nationwide survey in Japan.
The nationwide Kawasaki disease survey in Japan has been conducted biennially since 1970. The most recent survey was completed in 2019, obtaining information for patients who developed Kawasaki disease during 2017-2018. Survey respondents were hospitals specializing in pediatrics and those with ≥100 beds and a pediatric department throughout Japan, where patients with Kawasaki disease were eventually hospitalized.
The survey identified 32 528 patients with Kawasaki disease, which consisted of 15 164 (46.6%) in 2017 and 17 364 (53.4%) in 2018. The highest annual incidence rate was recorded in 2018 (359 per 100 000 children aged 0-4 years). After 1982, patients with ≤4 principal Kawasaki disease signs gradually increased, resulting in 6847 (21.1%) patients diagnosed during 2017-2018. Among the 30 784 patients receiving initial intravenous immunoglobulin administration, 6061 (19.7%) did not respond. Within 30 days of Kawasaki disease onset, 9.0% of patients were diagnosed with cardiac complications, and 2.6% of patients developed cardiac sequelae after the acute illness.
The annual number of patients developing Kawasaki disease in Japan increased from 1970 through 2018, whereas the proportion of patients with Kawasaki disease with cardiac complications decreased in the most recent 2 decades. Early diagnosis of Kawasaki disease as well as advances in initial treatments could explain these findings.
Outcomes in Kawasaki disease patients with coronary artery abnormalities at admission
2020, American Heart Journal
Citation Excerpt :
Friedman et al.24 followed patients with CA aneurysm for periods of up to 2 years, whereas ours reported results of the first assessment after 30 days of illness. Over 90% of patients with giant CA aneurysms at admission still exhibited giant aneurysms after 30 days in our study, which is consistent with previous studies including those conducted prior to the widespread establishment of standard IVIG treatment.13-16,21-24,32 Larger-sized CALs can take longer times to improve to normal size, and patients with giant CA aneurysms have been reported as being at higher risk for developing CA stenosis and subsequent myocardial infarction.16-18,21,24,32
Previous studies demonstrated that coronary artery lesions (CALs) resulting from Kawasaki disease (KD) can improve over time. However, limited information is available on sub-acute outcomes of CALs detected at admission during KD illness.
The nationwide Japanese KD survey contained substantial information on KD patients with CALs detected at admission and who received standard IVIG treatment within 10 days of disease onset. Coronary outcomes were evaluated by changes in CALs from admission to the first assessment at 30 days from disease onset in three categories: improved, unchanged, and progressed. Ordinal logistic regression analysis was performed to evaluate factors associated with the outcomes.
Of 2024 patients with CALs detected at admission, improved, unchanged, and progressed outcomes were found in 1548 (76.5%), 390 (19.3%), and 86 (4.2%), respectively. Over 80% of patients with coronary artery (CA) dilatations had improved outcome. Independent factors associated with worse outcomes were larger-size CALs (adjusted ORs [95% CIs]: CA aneurysm = 5.13 [3.65-7.22] and giant CA aneurysms = 7.49 [3.56-15.72] compared with CA dilatation, respectively), age ≥ 60 months (1.45 [1.08-1.94] compared with 12-59 months), recurrent KD (1.57 [1.07-2.29]), parental history of KD (2.23 [1.02-4.85]), and delayed admission (8-10 days from disease onset: 1.76 [1.21-2.57] compared with 1-4 days).
KD patients with larger CALs, ≥60 months old, and with recurrent status or parental history may require more rigorous treatment. In addition, delayed admission may result in worse coronary outcome, indicating that prompt diagnosis and treatment are required.
Using nomogram scores to predict the early regression of coronary artery aneurysms of Kawasaki disease
2023, Cardiology in the Young
Impact of Z score system on the management of coronary artery lesions in Kawasaki disease
2022, Cardiology in the Young

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⁺: Supported in part by a grant from the Heart and Stroke Foundation of Canada Research Fellowship.

^*: This article was prepared with the assistance of Hospital for Sick Children Medical Publications, Toronto, Ontario, Canada.

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Outcome of coronary artery aneurysms after Kawasaki disease+,*

J Pediatr

J Pediatr

Am J Cardiol

Am J Cardiol

J Pediatr

J Am Coll Cardiol

J Am Coll Cardiol

J Pediatr

Am J Cardiol

J Pediatr

Kawasaki disease: cardiac problems and management

Pediatr Rev

Mucocutaneous lymph node syndrome in the United States

Am J Dis Child

Results from an international survey of Kawasaki disease in 1979–82

Can J Cardiol