Giant coronary artery aneurysms in Kawasaki disease: the cost of a missed diagnosis

2013 ◽  
Vol 23 (4) ◽  
pp. 608-609
Author(s):  
Liliana Marta ◽  
Andreia Francisco ◽  
Rui Anjos

AbstractGiant coronary aneurysms secondary to Kawasaki disease are rare, but a very severe complication. Delayed diagnosis and appropriate treatment of the disease is a well-known risk factor for coronary aneurysms.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kenji Suda ◽  
Hiroshi Nishino ◽  
Yoshiyuki Kudo ◽  
Hironaga Yoshimoto ◽  
Shintaro Kishimoto ◽  
...  

Objective: We report four patients with incomplete Kawasaki disease (KD) successfully treated by antibiotics without intravenous immunoglobulin (IVIG) treatment with ≦ 5 days of fever but left with coronary artery lesion. Result: The patients were 2 babies and 2 young children age ranged from 2 months to 2 years old and 9 months. They showed fever and other signs of KD, but did not fulfill diagnostic criteria. The numbers of symptoms compatible with KD were 2 or 3 and included conjunctival injection, oral or lip injection, and eruption. Within 5 days, they became afebrile by antibiotics without IVIG treatment. However, they were noted to have coronary artery aneurysm (CAA) on day from 7 to 33 of illness. All patients had been placed on antithrombotic treatment including aspirin or aspirin plus warfarin and, fortunately, showed regression of CAA within 2 years. Conclusions: This case series indicates that there are patients with incomplete KD successfully treated by antibiotics without IVIG but left with CAA. These cases must be underdiagnosed if intentional echocardiographic examination are scheduled and might be left without appropriate treatment, antithrombotic treatment. Further collection of data of these patients is indispensable.


2020 ◽  
Vol 30 (12) ◽  
pp. 1821-1825 ◽  
Author(s):  
Etsuko Tsuda ◽  
Shuichi Yoneda ◽  
Yasuhide Asaumi ◽  
Atsuko Suzuki

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.


2018 ◽  
Vol 8 (3) ◽  
pp. 19-22
Author(s):  
Samir Duarte Ibrahim ◽  
Ana Laura Lopes Potente ◽  
Fernanda Pereira Maiolini

A doença de Kawasaki é caracterizada por uma vasculite aguda sistêmica que ocorre na grande maioria dos casos em crianças menores de cinco anos de idade. A doença tem curso autolimitado e tratamento baseado no uso de imunoglobulina intravenosa e salicilatos. A principal gravidade se dá pelas possíveis complicações, sendo a mais grave a formação de aneurismas de artérias coronárias. O presente trabalho visa relatar o caso de uma paciente que não recebeu o diagnóstico de doença de Kawasaki na infância. Na vida adulta, durante ecocardiograma diagnosticou-se uma hipocinesia de parede inferior e parte distal do septo inferior. Em posterior angiografia, verificou-se artéria coronária direita ectásica em toda a sua extensão. Trata-se de um caso raro devido à formação de aneurismas coronarianos não tratados, após anos de doença, e sua manifestação isquêmica.Palavras-Chave: Síndrome de linfonodos mucocutâneos; Doença das coronárias; Infarto do miocárdioABSTRACTKawasaki disease is characterized by the presence of acute systemic vasculitis that occurs in most of cases in children under five years of age. The disease has an auto-limited path and the treatment is based on intravenous immunoglobulin and salicylates. The main gravity is due to the possible complications, and the most serious is the formation of coronary artery aneurysms. The present work aims to report the case of a patient, who did not receive the diagnosis of Kawasaki disease in childhood. In adulthood, an inferior wall hypokinesia and a distal part of the inferior septum were diagnosed by echocardiogram. In a subsequent angiography it was verified a ectasia right coronary artery throughout its extension. This is a rare case due to the formation of untreated coronary aneurysms after years of illness associated with ischemic manifestations.Keywords: Mucocutaneous lymph node syndrome;  Myocardial infarction; Coronary disease


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
William Sarmiento-Robles ◽  
Luis M Garrido-Garcia

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.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Michael Khoury ◽  
Michael A Portman ◽  
Cedric Manlhiot ◽  
Anne Fournier ◽  
Rejane F Dillenburg ◽  
...  

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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